Human Disease Model Report: RNA repeat diseases

General Information

Name

RNA repeat diseases

FlyBase ID

FBhh0000059

Disease Ontology Term

neurodegenerative disease

Parent Disease

OMIM

Overview

For a number of nucleotide-repeat-expansion diseases, expansions occur within non-coding regions such as UTRs, introns, promoters, or within non-coding genes. The pathological effects of such non-coding expansions appear to be complex and varied. Frequently, non-coding expansions appear to result in repeat-containing transcripts with gain-of-function RNA toxicity. It at least one case, a profound impact on gene expression has been demonstrated (see FBhh0000123). In some cases, non-coding expansions appear to be translated into toxic peptides by repeat-associated non-AUG translation (RAN); some aspects of this phenomenon have been investigated in Drosophila human disease models (see FBhh0000137 and FBhh0000024).

This report includes model systems developed in Drosophila using UAS constructs to drive synthetic constructs of nucleotide repeats of variable composition and length. Synthetic alleles are designated with the prefix "Zzzz" in FlyBase (see the 'Alleles Reported to Model Human Disease' section, below); see also the allele report for CG9650^CTG-240.4 (FBal0243220). See the 'Related Diseases' section, below, for a list of RNA repeat diseases characterized in flies.

Although RNA-mediated mechanisms of neurotoxicity may also play a role in diseases associated with polypeptide repeats, expanded CAG repeats within polyglutamine-containing proteins have been shown to be due (at least primarily) to the repeat-containing protein. Results in Drosophila addressing polyglutamine models are described in disease model reports 'polyglutamine diseases, polyQ models' (FBhh0000001), 'polyglutamine diseases' (FBhh0000218), and related disease models listed in these reports.

[updated Sep. 2022 by FlyBase; FBrf0222196]

Disease Summary Information

Disease Summary: RNA repeat diseases

OMIM report

Human gene(s) implicated

Symptoms and phenotype

Genetics

A broad grouping of diseases caused by genes containing nucleotide repeat expansions (most commonly, but not exclusively, nucleotide triplet repeats); expansions may be located in exons, UTRs, or introns.

(Chan, 2014)

Cellular phenotype and pathology

Molecular information

RNA-mediated mechanisms of neurotoxicity appear to play a significant role in the pathogenesis of repeat expansion diseases, probably in conjunction with the protein-mediated pathways. Multiple different mechanisms, such as the alteration of gene expression levels and splicing patterns, the generation of small RNAs, the induction of nucleolar stress, the promotion of bi-directional transcription and repeat-associated non-ATG translation of the disease locus, the activation of apoptotic signaling, and the sequestration of cellular components to RNA foci, have been shown for different repeat expansion diseases.

(Chan, 2014)

Non-coding expansion disorders typically involve large expansions (from ~100 to 1000 copies), which reside in the non-coding regions of genes, such as promoters, introns or untranslated regions (UTRs). Non-coding expansions typically result in repeat-containing transcripts with gain-of-function RNA toxicity. However, even non-coding expansions can still be translated into toxic peptides by repeat-associated non-ATG translation.

(Koon and Chan, 2017)

External links

Disease synonyms

microsatellite-expansion diseases

non-coding expansion disorders

noncoding repeat expansion diseases

nucleotide repeat expansion diseases

READs

repeat-expansion associated diseases

repeat expansion disorder

untranslated repeat diseases

Related Diseases

Related human health report(s)

fragile-X-related syndromes

fragile X tremor/ataxia syndrome

Friedreich ataxia 1

frontotemporal dementia and/or amyotrophic lateral sclerosis 1

myotonic dystrophy 1

myotonic dystrophy 2

neuronal intranuclear inclusion disease

polyalanine diseases

polyglutamine diseases

polyglutamine diseases, polyQ models

spinocerebellar ataxia 8

spinocerebellar ataxia 10

spinocerebellar ataxia 12

spinocerebellar ataxia 31

Ortholog Information

Human gene(s) in FlyBase

Other mammalian ortholog(s) used

D. melanogaster Gene Information (0)

Other Genes Used: Viral, Bacterial, Synthetic (6)

Summary of Physical Interactions (0 groups)

Alleles Reported to Model Human Disease (Disease Ontology) (52 alleles)

Zzzz\GGGGCC

Models Based on Experimental Evidence ( 31 )

Allele

Disease

Evidence

References

Zzzz\GGGGCC^RO-36.UAS

DOES NOT model amyotrophic lateral sclerosis

CEA

(Mizielinska et al., 2014)

DOES NOT model frontotemporal dementia

CEA

(Mizielinska et al., 2014)

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Kim et al., 2021)

Zzzz\GGGGCC^RO-108.UAS

DOES NOT model frontotemporal dementia

CEA

(Mizielinska et al., 2014)

DOES NOT model amyotrophic lateral sclerosis

CEA

(Mizielinska et al., 2014)

DOES NOT model frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Moens et al., 2018)

Zzzz\GGGGCC^RO-288.UAS

DOES NOT model amyotrophic lateral sclerosis

CEA

(Mizielinska et al., 2014)

DOES NOT model frontotemporal dementia

CEA

(Mizielinska et al., 2014)

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Park et al., 2020)

Zzzz\GGGGCC^3.UAS.EGFP

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Ramesh et al., 2020)

Zzzz\GGGGCC^30.UAS.EGFP

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEC

(Pun et al., 2022, Kim et al., 2019, Anderson et al., 2018)

CEA

(Lee et al., 2024, Jhan et al., 2021, Jiao et al., 2021, Maor-Nof et al., 2021, Zaepfel et al., 2021, Cunningham et al., 2020, Han et al., 2020, Kankel et al., 2020, Lee et al., 2020, Ortega et al., 2020, Ramesh et al., 2020, Wen et al., 2020, He et al., 2019, Zhang et al., 2015)

model of amyotrophic lateral sclerosis

CEA

(Xu et al., 2013)

model of frontotemporal dementia

CEA

(Xu et al., 2013)

model of neurodegenerative disease

CEA

(Celona et al., 2017)

Zzzz\GGGGCC^3.UAS

DOES NOT model amyotrophic lateral sclerosis

CEA

(Hautbergue et al., 2017, Mizielinska et al., 2014)

DOES NOT model frontotemporal dementia

CEA

(Mizielinska et al., 2014)

Zzzz\GGGGCC^103.UAS

model of amyotrophic lateral sclerosis

CEA

(Mizielinska et al., 2014)

model of frontotemporal dementia

CEA

(Mizielinska et al., 2014)

Zzzz\GGGGCC^58.UAS.EGFP

model of amyotrophic lateral sclerosis

CEA

(Freibaum et al., 2015)

model of frontotemporal dementia

CEA

(Freibaum et al., 2015)

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEC

(Cheng et al., 2019)

CEA

(Bennett et al., 2023, Ramesh et al., 2020)

Zzzz\GGGGCC^48.UAS

model of neurodegenerative disease

CEA

(Schweizer Burguete et al., 2015)

Zzzz\GGGGCC^{32.UAS.DsRed2}

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Solomon et al., 2018)

Zzzz\GGGGCC^{64.UAS.DsRed2}

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Solomon et al., 2018)

Zzzz\GGGGCC^{38.UAS.DsRed2}

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Solomon et al., 2018)

Zzzz\GGGGCC^{44.LDS.UAS.GR-GFP}

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Gotoh et al., 2024, Lee et al., 2024, Fujino et al., 2023, Cunningham et al., 2020)

CEC

(Goodman et al., 2019)

Zzzz\GGGGCC^29.UAS

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Kramer et al., 2016)

Zzzz\GGGGCC^49.UAS

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Goodman et al., 2019, Mordes et al., 2018, Kramer et al., 2016)

Zzzz\GGGGCC^{GFP-iC242.UAS}

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(He et al., 2020)

Zzzz\GGGGCC^{0+G4C2.UAS.EGFP}

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(He et al., 2020)

Zzzz\GGGGCC^{1+G4C2.UAS.EGFP}

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(He et al., 2020)

Zzzz\GGGGCC^{2+G4C2.UAS.EGFP}

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(He et al., 2020)

Zzzz\GGGGCC^69.CT.UAS

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(He et al., 2020)

Zzzz\GGGGCC^69.ΔCT.UAS

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(He et al., 2020)

Zzzz\GGGGCC^{1+G4C2x28.UAS.EGFP}

model of neurodegenerative disease

CEA

(Green et al., 2022)

Zzzz\GGGGCC^42.UAS

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Fujino et al., 2023)

Zzzz\GGGGCC^89.UAS

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Fujino et al., 2023, Taminato et al., 2023)

Zzzz\GGGGCC^107.AS.UAS

DOES NOT model frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Moens et al., 2018)

Zzzz\GGGGCC^EGFP.i-106RO

DOES NOT model frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Moens et al., 2018)

Zzzz\GGGGCC^{EGFP.i-108RO.AS}

DOES NOT model frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Moens et al., 2018)

Zzzz\GGGGCC^800RO.UAS

DOES NOT model frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Moens et al., 2018)

Zzzz\GGGGCC^1000RO.UAS

DOES NOT model frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Moens et al., 2018)

Zzzz\GGGGCC^{2000-5000RO.UAS}

DOES NOT model frontotemporal dementia and/or amyotrophic lateral sclerosis 1

CEA

(Moens et al., 2018)

Modifiers Based on Experimental Evidence ( 11 )

Allele

Disease

Interaction

References

Zzzz\GGGGCC^30.UAS.EGFP

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

is ameliorated by AstC-R1^JF02656

(Pun et al., 2022)

is ameliorated by ERR^JF02431

(Pun et al., 2022)

is ameliorated by Eip78C^JF02258

(Pun et al., 2022)

is ameliorated by Octα2R^HMC03079

(Pun et al., 2022)

is ameliorated by Pp2B-14D^JF01949

(Pun et al., 2022)

is ameliorated by Ptp69D^JF03399

(Pun et al., 2022)

is ameliorated by Shab^JF01823

(Pun et al., 2022)

is ameliorated by p53^GL01032

(Maor-Nof et al., 2021)

is ameliorated by p53^GL01220

(Maor-Nof et al., 2021)

is ameliorated by p53^HMS02286

(Maor-Nof et al., 2021)

is exacerbated by eRF1^GD4704

(Ortega et al., 2020)

is exacerbated by Art1^KK101196

(Ortega et al., 2020)

is exacerbated by Tnpo-SR^GD9833

(Ortega et al., 2020)

is exacerbated by e(y)2^KK102036

(Ortega et al., 2020)

is ameliorated by Hsap\SIGMAR1^UAS.cCa

(Lee et al., 2020)

is ameliorated by Gle1^HMC03626

(Kankel et al., 2020)

is exacerbated by Hsap\MAPT^UAS.cWa

(Wen et al., 2020)

is ameliorated by tau^KO

(Wen et al., 2020)

is ameliorated by Atg18a^HMS01193

(Cunningham et al., 2020)

is exacerbated by Atg18b^HMS01194

(Cunningham et al., 2020)

is exacerbated by Atg2^HMS01198

(Cunningham et al., 2020)

is exacerbated by Atg2^JF02786

(Cunningham et al., 2020)

is exacerbated by Atg3^EY08396

(Cunningham et al., 2020)

is exacerbated by Atg3^HMS01348

(Cunningham et al., 2020)

is exacerbated by Atg4b^P0997

(Cunningham et al., 2020)

is ameliorated by Atg5^JF02703

(Cunningham et al., 2020)

is ameliorated by Atg6^HMS01483

(Cunningham et al., 2020)

is ameliorated by Atg6^JF02897

(Cunningham et al., 2020)

is ameliorated by Atg7^HMS01358

(Cunningham et al., 2020)

is ameliorated by Atg8a^HMS01328

(Cunningham et al., 2020)

is ameliorated by Atg8a^JF02895

(Cunningham et al., 2020)

is exacerbated by Mitf^HMS02712

(Cunningham et al., 2020)

is ameliorated by Mitf^SI1-RES

(Cunningham et al., 2020)

is ameliorated by Rab7^UASp.YFP

(Cunningham et al., 2020)

is exacerbated by RanGAP^JF03244

(Cunningham et al., 2020, Zhang et al., 2015)

is exacerbated by Snap29^B6-21

(Cunningham et al., 2020)

is ameliorated by Trpml^UAS.Tag:MYC

(Cunningham et al., 2020)

is exacerbated by bchs^UAS.Tag:HA

(Cunningham et al., 2020)

is ameliorated by emb^JF01311

(Cunningham et al., 2020)

is ameliorated by ref(2)P^HMS00551

(Cunningham et al., 2020)

is exacerbated by ref(2)P^UAS.Tag:HA

(Cunningham et al., 2020)

is ameliorated by Atg1^UAS.cSa

(Cunningham et al., 2020)

is ameliorated by Atg101^HMS01349

(Cunningham et al., 2020)

is exacerbated by Atg13^HMS02028

(Cunningham et al., 2020)

is ameliorated by Atg14^HMC04086

(Cunningham et al., 2020)

is exacerbated by Atg14^HMS02025

(Cunningham et al., 2020)

is exacerbated by Atg16^HMJ22265

(Cunningham et al., 2020)

is exacerbated by Atg16^HMS01347

(Cunningham et al., 2020)

is exacerbated by Atg17^HMS01611

(Cunningham et al., 2020)

is exacerbated by Mhc^KK102402

(Kim et al., 2019)

is exacerbated by Mhc^HMS01471

(Kim et al., 2019)

is exacerbated by Mhc^JF02069

(Kim et al., 2019)

is ameliorated by RanGAP^Sd

(Zhang et al., 2015)

is ameliorated by RanGAP^UAS.Tag:HA

(Zhang et al., 2015)

is ameliorated by CG8173^JF01161

(Jiao et al., 2021)

is ameliorated by Myb^JF02135

(Jiao et al., 2021)

is ameliorated by Top2^GL00338

(Jiao et al., 2021)

is ameliorated by Top2^JF01300

(Jiao et al., 2021)

is ameliorated by Hsap\MATR3^{ΔRRM1.UAS.Tag:FLAG}

(Ramesh et al., 2020)

is ameliorated by Hsap\MATR3^{ΔZNF1.UAS.Tag:FLAG}

(Ramesh et al., 2020)

is ameliorated by Hsap\MATR3^{ΔZNF2.UAS.Tag:FLAG}

(Ramesh et al., 2020)

is ameliorated by Hsap\MATR3^UAS.Tag:FLAG

(Ramesh et al., 2020)

is exacerbated by Upf1^GL01485

(Zaepfel et al., 2021)

is exacerbated by Upf1^HMC05537

(Zaepfel et al., 2021)

is ameliorated by Upf1^UASp.GFP

(Zaepfel et al., 2021)

is ameliorated by Rel^GD1199

(Han et al., 2020)

is ameliorated by tup^HMC03317

(Han et al., 2020)

is exacerbated by Rhau^UAS.Tag:HA

(Lee et al., 2024)

model of amyotrophic lateral sclerosis

is ameliorated by Pur-α^UAS.Tag:FLAG

(Xu et al., 2013)

model of frontotemporal dementia

is ameliorated by Pur-α^UAS.Tag:FLAG

(Xu et al., 2013)

model of neurodegenerative disease

is ameliorated by Mmus\Zfp106^{UAS.Tag:FLAG,Tag:StrepII}

(Celona et al., 2017)

exacerbates tauopathy

modeled by Hsap\MAPT^UAS.cWa

(He et al., 2019)

modeled by Hsap\MAPT^R406W.UAS

(He et al., 2019)

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 2

is ameliorated by HDAC6^JF01501

(Kankel et al., 2020)

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 4

is ameliorated by Hsp110^d03562

(Kankel et al., 2020)

is ameliorated by Rala^HMS01365

(Kankel et al., 2020)

is ameliorated by Rala^S25N.UAS

(Kankel et al., 2020)

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 3

is ameliorated by futsch^EP1419

(Kankel et al., 2020)

Zzzz\GGGGCC^36.UAS

model of amyotrophic lateral sclerosis

is ameliorated by SF2^GD12097

(Hautbergue et al., 2017)

is NOT ameliorated by Ref1^KK109076

(Hautbergue et al., 2017)

is NOT ameliorated by Ref1^GD4683

(Hautbergue et al., 2017)

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

is exacerbated by e(y)2^KK102036

(Ortega et al., 2020)

is exacerbated by eRF1^GD4704

(Ortega et al., 2020)

is exacerbated by Art1^KK101196

(Ortega et al., 2020)

is exacerbated by CCT8^KK100186

(Ortega et al., 2020)

is ameliorated by SF2^GD12097

(Castelli et al., 2021, Ortega et al., 2020)

is exacerbated by Tnpo-SR^GD9833

(Ortega et al., 2020)

is ameliorated by ref(2)P^HMS00551

(Cunningham et al., 2020)

is ameliorated by ref(2)P^HMS00938

(Cunningham et al., 2020)

is exacerbated by ref(2)P^UAS.Tag:HA

(Cunningham et al., 2020)

is ameliorated by eIF1A^UAS.ORF

(Moens et al., 2019)

is ameliorated by Akt^{T342D.S505D.GMR}

(Atilano et al., 2021)

is exacerbated by InR^K1409A.UAS

(Atilano et al., 2021)

is ameliorated by SK^MI09020

(Castelli et al., 2021)

is ameliorated by SK^MI10378

(Castelli et al., 2021)

is ameliorated by Hsap\MATR3^UAS.Tag:FLAG

(Ramesh et al., 2020)

is ameliorated by Pka-C1^KK108966

(Licata et al., 2022)

is ameliorated by Pka-C1^VSH330111

(Licata et al., 2022)

is exacerbated by LanB1^EP-600

(Catterson et al., 2023)

is exacerbated by chk^{UAS.ORF.GW.Tag:HA}

(Xu et al., 2023)

is ameliorated by bumpel^{UAS.ORF.GW.Tag:HA}

(Xu et al., 2023)

Zzzz\GGGGCC^58.UAS.EGFP

model of amyotrophic lateral sclerosis

is exacerbated by Nup50^GD9739

(Freibaum et al., 2015)

is exacerbated by Nup50^KG09557

(Freibaum et al., 2015)

is exacerbated by Nup50^KK108150

(Freibaum et al., 2015)

is exacerbated by Ran^{T24N.UASp.Tag:HA}

(Freibaum et al., 2015)

is exacerbated by Nup153^GD10636

(Freibaum et al., 2015)

is exacerbated by Nup153^KK110135

(Freibaum et al., 2015)

is exacerbated by CG9775^KK110941

(Freibaum et al., 2015)

is exacerbated by Tnpo^KK108990

(Freibaum et al., 2015)

is ameliorated by Ref1⁰²²⁶⁷

(Freibaum et al., 2015)

is exacerbated by Gle1^JF01431

(Freibaum et al., 2015)

is exacerbated by sbr^GD14541

(Freibaum et al., 2015)

is exacerbated by Cbp20^KK109448

(Freibaum et al., 2015)

is exacerbated by sbr^KK100882

(Freibaum et al., 2015)

is exacerbated by Cbp80^KK100796

(Freibaum et al., 2015)

is exacerbated by sbr^HM05135

(Freibaum et al., 2015)

is exacerbated by Cbp80^GD12110

(Freibaum et al., 2015)

is exacerbated by Ars2^KK109705

(Freibaum et al., 2015)

is exacerbated by Ars2^HMS00626

(Freibaum et al., 2015)

is exacerbated by Ars2^GL00079

(Freibaum et al., 2015)

is exacerbated by Rrp6^KK100590

(Freibaum et al., 2015)

is exacerbated by Rrp40^GD10414

(Freibaum et al., 2015)

is ameliorated by Nup98-96^KK100388

(Freibaum et al., 2015)

is ameliorated by Nup98-96^GD6897

(Freibaum et al., 2015)

is ameliorated by Nup107^KK108047

(Freibaum et al., 2015)

is ameliorated by Nup107^GD12024

(Freibaum et al., 2015)

is ameliorated by Nup160^KK104805

(Freibaum et al., 2015)

is exacerbated by emb^JF01311

(Freibaum et al., 2015)

is exacerbated by LBR^HMC02426

(Freibaum et al., 2015)

model of frontotemporal dementia

is exacerbated by Nup50^GD9739

(Freibaum et al., 2015)

is exacerbated by Nup50^KG09557

(Freibaum et al., 2015)

is exacerbated by Nup50^KK108150

(Freibaum et al., 2015)

is exacerbated by Ran^{T24N.UASp.Tag:HA}

(Freibaum et al., 2015)

is exacerbated by Nup153^GD10636

(Freibaum et al., 2015)

is exacerbated by LBR^HMC02426

(Freibaum et al., 2015)

is exacerbated by Nup153^KK110135

(Freibaum et al., 2015)

is exacerbated by Tnpo^KK108990

(Freibaum et al., 2015)

is ameliorated by Ref1⁰²²⁶⁷

(Freibaum et al., 2015)

is exacerbated by Gle1^JF01431

(Freibaum et al., 2015)

is exacerbated by CG9775^KK110941

(Freibaum et al., 2015)

is exacerbated by Cbp20^KK109448

(Freibaum et al., 2015)

is exacerbated by sbr^GD14541

(Freibaum et al., 2015)

is exacerbated by Cbp80^KK100796

(Freibaum et al., 2015)

is exacerbated by sbr^KK100882

(Freibaum et al., 2015)

is exacerbated by Cbp80^GD12110

(Freibaum et al., 2015)

is exacerbated by Ars2^KK109705

(Freibaum et al., 2015)

is exacerbated by Ars2^HMS00626

(Freibaum et al., 2015)

is exacerbated by Ars2^GL00079

(Freibaum et al., 2015)

is exacerbated by Rrp6^KK100590

(Freibaum et al., 2015)

is exacerbated by sbr^HM05135

(Freibaum et al., 2015)

is exacerbated by Rrp40^GD10414

(Freibaum et al., 2015)

is ameliorated by Nup98-96^KK100388

(Freibaum et al., 2015)

is ameliorated by Nup98-96^GD6897

(Freibaum et al., 2015)

is ameliorated by Nup107^KK108047

(Freibaum et al., 2015)

is ameliorated by Nup107^GD12024

(Freibaum et al., 2015)

is ameliorated by Nup160^KK104805

(Freibaum et al., 2015)

is exacerbated by emb^JF01311

(Freibaum et al., 2015)

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

is exacerbated by bel^EY08943

(Cheng et al., 2019)

is exacerbated by bel^GL00205

(Cheng et al., 2019)

is exacerbated by bel^JF02884

(Cheng et al., 2019)

is ameliorated by Hsap\MATR3^UAS.Tag:FLAG

(Ramesh et al., 2020)

is ameliorated by Hsap\SETX^{L389S.UAS.Tag:FLAG}

(Bennett et al., 2023)

is ameliorated by Hsap\SETX^UAS.Tag:FLAG

(Bennett et al., 2023)

Zzzz\GGGGCC^48.UAS

model of neurodegenerative disease

is ameliorated by Fmr1^HMS00248

(Schweizer Burguete et al., 2015)

is exacerbated by Fmr1^UAS.cWa

(Schweizer Burguete et al., 2015)

is ameliorated by orb2^JF02376

(Schweizer Burguete et al., 2015)

is exacerbated by orb2^UAS.cUa

(Schweizer Burguete et al., 2015)

Zzzz\GGGGCC^{38.UAS.DsRed2}

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

is exacerbated by TBPH^ΔNLS

(Solomon et al., 2018)

Zzzz\GGGGCC^{44.LDS.UAS.GR-GFP}

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

is ameliorated by eIF5^HMS00159

(Gotoh et al., 2024)

is ameliorated by eIF5^KK102299

(Gotoh et al., 2024)

is ameliorated by ref(2)P^HMS00551

(Cunningham et al., 2020)

is exacerbated by ref(2)P^UAS.Tag:HA

(Cunningham et al., 2020)

is ameliorated by eIF4B^HMS04503

(Goodman et al., 2019)

is ameliorated by eIF4B^VSH330010

(Goodman et al., 2019)

is ameliorated by eIF4H1^HMS04504

(Goodman et al., 2019)

is ameliorated by eIF4H1^KK108805

(Goodman et al., 2019)

Zzzz\GGGGCC^29.UAS

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

is ameliorated by spt4^HMS00685

(Kramer et al., 2016)

Zzzz\GGGGCC^49.UAS

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

is ameliorated by Atu^GD8296

(Goodman et al., 2019)

is ameliorated by Atu^KK104959

(Goodman et al., 2019)

is ameliorated by atms^GD9782

(Goodman et al., 2019)

is ameliorated by atms^KK100080

(Goodman et al., 2019)

is ameliorated by hyx^GD12640

(Goodman et al., 2019)

is ameliorated by Ctr9^HMS00619

(Goodman et al., 2019)

is ameliorated by Ctr9^KK101412

(Goodman et al., 2019)

is ameliorated by Rtf1^HMS00168

(Goodman et al., 2019)

is ameliorated by hyx^HMC03494

(Goodman et al., 2019)

is ameliorated by Rtf1^KK101209

(Goodman et al., 2019)

is ameliorated by spt4^HMS00685

(Kramer et al., 2016)

is exacerbated by Hsf^+t8

(Mordes et al., 2018)

Zzzz\GGGGCC^{1+G4C2x28.UAS.EGFP}

model of neurodegenerative disease

is ameliorated by DENR^GD17852

(Green et al., 2022)

is ameliorated by DENR^KK109336

(Green et al., 2022)

is ameliorated by eIF2A^HMC03050

(Green et al., 2022)

is ameliorated by eIF2D^HMS00958

(Green et al., 2022)

Zzzz\GGGGCC^42.UAS

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

is ameliorated by Hsap\SF3B3^UAS.cFa

(Fujino et al., 2023)

is exacerbated by Hsap\SFPQ^UAS.cFa

(Fujino et al., 2023)

is ameliorated by Hsap\FUS^UAS.cIa

(Fujino et al., 2023)

is ameliorated by Hsap\HNRNPA1^UAS.cFa

(Fujino et al., 2023)

is ameliorated by Hsap\HNRNPA2B1^UAS.cFa

(Fujino et al., 2023)

is exacerbated by Hsap\HNRNPK^UAS.cFa

(Fujino et al., 2023)

is ameliorated by Hsap\HNRNPL^UAS.cFa

(Fujino et al., 2023)

is ameliorated by Hsap\HNRNPR^UAS.cFa

(Fujino et al., 2023)

is ameliorated by Hsap\IGF2BP1^UAS.cFa

(Fujino et al., 2023)

is ameliorated by Hsap\SAFB2^UAS.cFa

(Fujino et al., 2023)

Zzzz\GGGGCC^89.UAS

model of frontotemporal dementia and/or amyotrophic lateral sclerosis 1

is ameliorated by Hsap\SF3B3^UAS.cFa

(Fujino et al., 2023, Taminato et al., 2023)

is exacerbated by Hsap\SFPQ^UAS.cFa

(Fujino et al., 2023)

is exacerbated by caz²

(Fujino et al., 2023)

is exacerbated by caz^KK107486

(Fujino et al., 2023)

is ameliorated by Hsap\DDX17^UAS.cFa

(Fujino et al., 2023)

is ameliorated by Hsap\DDX3X^UAS.cFa

(Fujino et al., 2023)

is ameliorated by Hsap\DDX5^UAS.cFa

(Fujino et al., 2023)

is ameliorated by Hsap\DHX36^UAS.cFa

(Fujino et al., 2023)

is ameliorated by Hsap\DHX9^UAS.cFa

(Fujino et al., 2023)

is ameliorated by Hsap\EWSR1^UAS.cCa

(Fujino et al., 2023)

is ameliorated by Hsap\FUS^UAS.cIa

(Fujino et al., 2023)

is ameliorated by Hsap\HNRNPA1^UAS.cFa

(Fujino et al., 2023)

is ameliorated by Hsap\HNRNPA2B1^UAS.cFa

(Fujino et al., 2023, Taminato et al., 2023)

is exacerbated by Hsap\HNRNPK^UAS.cFa

(Fujino et al., 2023)

is ameliorated by Hsap\HNRNPL^UAS.cFa

(Fujino et al., 2023)

is ameliorated by Hsap\HNRNPR^UAS.cFa

(Fujino et al., 2023, Taminato et al., 2023)

is ameliorated by Hsap\IGF2BP1^UAS.cFa

(Fujino et al., 2023, Taminato et al., 2023)

is ameliorated by Hsap\SAFB2^UAS.cFa

(Fujino et al., 2023)

is exacerbated by Hrb87F^KK108162

(Taminato et al., 2023)

is ameliorated by Hsap\HNRNPA3^UAS.cTa

(Taminato et al., 2023)

Zzzz\CCG

Models Based on Experimental Evidence ( 1 )

Allele

Disease

Evidence

References

Zzzz\CCG^90.UAS.EGFP

model of fragile X-associated tremor/ataxia syndrome

CEA

(Sofola et al., 2007)

Modifiers Based on Experimental Evidence ( 1 )

Allele

Disease

Interaction

References

Zzzz\CCG^90.UAS.EGFP

ameliorates fragile X-associated tremor/ataxia syndrome

modeled by Zzzz\CGG^90.UAS.EGFP

(Sofola et al., 2007)

model of fragile X-associated tremor/ataxia syndrome

is ameliorated by Zzzz\CGG^90.UAS.EGFP

(Sofola et al., 2007)

Zzzz\CGG

Models Based on Experimental Evidence ( 4 )

Allele

Disease

Evidence

References

Zzzz\CGG^{100.5'UTR.UAS.GFP}

model of fragile X-associated tremor/ataxia syndrome

CEA

(Oh et al., 2015)

Zzzz\CGG^90.UAS.EGFP

model of fragile X-associated tremor/ataxia syndrome

CEA

(Bhat et al., 2021, Kong et al., 2019, Linsalata et al., 2019, Weber et al., 2016, Oh et al., 2015, Galloway et al., 2014, Tan et al., 2012, Sofola et al., 2007, Sofola et al., 2007)

model of fragile X syndrome

CEA

(Zhang et al., 2018, Todd et al., 2013)

model of neurodegenerative disease

CEA

(Green et al., 2022, Peng et al., 2021, He et al., 2014, Todd et al., 2013, Qurashi et al., 2012, Tan et al., 2012, Todd et al., 2010, Jin et al., 2003)

model of primary ovarian insufficiency

CEA

(Trevino et al., 2021)

Zzzz\CGG^60.UAS.EGFP

model of neurodegenerative disease

CEA

(Qurashi et al., 2012)

model of fragile X-associated tremor/ataxia syndrome

CEA

(Bhat et al., 2021, Tan et al., 2012)

Zzzz\CGG^103.UAS.EGFP

model of fragile X-associated tremor/ataxia syndrome

CEA

(Singh et al., 2021)

Modifiers Based on Experimental Evidence ( 4 )

Allele

Disease

Interaction

References

Zzzz\CGG^{100.5'UTR.UAS.GFP}

model of fragile X-associated tremor/ataxia syndrome

is exacerbated by Prosβ6^1.B.UAS

(Oh et al., 2015)

is exacerbated by Prosβ2^1.UAS

(Oh et al., 2015)

Zzzz\CGG^90.UAS.EGFP

ameliorates fragile X-associated tremor/ataxia syndrome

modeled by Zzzz\CCG^90.UAS.EGFP

(Sofola et al., 2007)

model of fragile X-associated tremor/ataxia syndrome

is ameliorated by Zzzz\CCG^90.UAS.EGFP

(Sofola et al., 2007)

is ameliorated by Hsap\TARDBP^UAS.cLa

(Galloway et al., 2014)

is exacerbated by TBPH^GD6943

(Galloway et al., 2014)

is ameliorated by Hsap\CELF1^UAS.cdHa

(Sofola et al., 2007)

is ameliorated by Hsap\HNRNPA2B1^UAS.cSa

(Sofola et al., 2007)

is ameliorated by Hrb87F^UAS.cSa

(Sofola et al., 2007)

is ameliorated by Hrb98DE^UAS.cSa

(Sofola et al., 2007)

is exacerbated by Prosβ6^1.B.UAS

(Oh et al., 2015)

is exacerbated by Prosβ2^1.UAS

(Oh et al., 2015)

is ameliorated by Hsc70-4^UAS.cEa

(Oh et al., 2015)

is ameliorated by eIF1^GLC01849

(Linsalata et al., 2019)

is exacerbated by Rhau^EY09315

(Linsalata et al., 2019)

is exacerbated by RpS25^GD10582

(Linsalata et al., 2019)

is exacerbated by RpS25^KK107958

(Linsalata et al., 2019)

is ameliorated by SF2^HM05199

(Linsalata et al., 2019)

is ameliorated by SF2^HMS00358

(Linsalata et al., 2019)

is ameliorated by bel⁵

(Linsalata et al., 2019)

is ameliorated by bel⁶

(Linsalata et al., 2019)

is ameliorated by bel^GD1324

(Linsalata et al., 2019)

is ameliorated by bel^GL00057

(Linsalata et al., 2019)

is ameliorated by bel^GL00205

(Linsalata et al., 2019)

is ameliorated by bel^JF02884

(Linsalata et al., 2019)

is ameliorated by bel^L4740

(Linsalata et al., 2019)

is ameliorated by bel^S074407

(Linsalata et al., 2019)

is ameliorated by bel^cap-1

(Linsalata et al., 2019)

is ameliorated by bgcn^GD10012

(Linsalata et al., 2019)

is ameliorated by bgcn^KK106752

(Linsalata et al., 2019)

is ameliorated by eIF1^EY02210

(Linsalata et al., 2019)

is ameliorated by eIF1^HMC04556

(Linsalata et al., 2019)

is exacerbated by eIF1^HMC04556

(Linsalata et al., 2019)

is exacerbated by eIF1^KK109232

(Linsalata et al., 2019)

is exacerbated by eIF1A⁶⁴⁵

(Linsalata et al., 2019)

is exacerbated by eIF1A^GD10618

(Linsalata et al., 2019)

is exacerbated by eIF1A^JF01698

(Linsalata et al., 2019)

is exacerbated by eIF1A^JF02475

(Linsalata et al., 2019)

is exacerbated by eIF1A^KK108681

(Linsalata et al., 2019)

is exacerbated by eIF2α^815-29

(Linsalata et al., 2019)

is exacerbated by eIF2α^GD1430

(Linsalata et al., 2019)

is exacerbated by eIF2α^KK100282

(Linsalata et al., 2019)

is exacerbated by eIF3b^EY14430

(Linsalata et al., 2019)

is ameliorated by eIF4B^GD7103

(Linsalata et al., 2019)

is exacerbated by eIF4B^GD7103

(Linsalata et al., 2019)

is ameliorated by eIF4H1^KK108805

(Linsalata et al., 2019)

is ameliorated by eIF4H2^KK103972

(Linsalata et al., 2019)

is ameliorated by CG4306^HMC06152

(Kong et al., 2019)

is exacerbated by CG8353^HMS03373

(Kong et al., 2019)

is exacerbated by ChAT^HMC05021

(Kong et al., 2019)

is exacerbated by Pect^HMJ30278

(Kong et al., 2019)

is exacerbated by Ppat-Dpck^HMC04615

(Kong et al., 2019)

is exacerbated by Sk2^HMS03001

(Kong et al., 2019)

is exacerbated by ras^JF01445

(Kong et al., 2019)

is exacerbated by schlank^JF02502

(Kong et al., 2019)

is ameliorated by Pur-α^RI.RII.UAS

(Weber et al., 2016)

is ameliorated by Pur-α^UAS.cWa

(Weber et al., 2016)

is exacerbated by lncRNA:flam^BG02658

(Tan et al., 2012)

is exacerbated by lncRNA:flam^KG00476

(Tan et al., 2012)

is ameliorated by Drep2^ex13

(Bhat et al., 2021)

is ameliorated by bsk¹

(Bhat et al., 2021)

is ameliorated by Pur-α^UAS.Tag:FLAG

(Jin et al., 2007)

model of neurodegenerative disease

is ameliorated by Hsc70-4^UAS.cEa

(Jin et al., 2003)

is exacerbated by Hsc70-4^K71S.UAS

(Jin et al., 2003)

is ameliorated by GIIIspla2^EP1516

(Qurashi et al., 2012)

is ameliorated by GIIIspla2^GD17243

(Qurashi et al., 2012)

is exacerbated by HDAC6^RNAi.UAS

(Todd et al., 2010)

is ameliorated by HDAC3^{UAS.Tag:V5,Tag:polyHis}

(Todd et al., 2010)

is ameliorated by HDAC6^{UAS.Tag:V5,Tag:polyHis}

(Todd et al., 2010)

is ameliorated by HDAC11^{UAS.Tag:V5,Tag:polyHis}

(Todd et al., 2010)

is exacerbated by HDAC11^RNAi.UAS

(Todd et al., 2010)

is exacerbated by vimar^k16722

(Tan et al., 2012)

is exacerbated by Drep2^KG02396

(Tan et al., 2012)

is ameliorated by Avic\GFP^{EGFP.Scer\UAS.P\T.mir-276aSP}

(Tan et al., 2012)

is ameliorated by Drep2^d00223

(Tan et al., 2012)

is exacerbated by mir-277^UAS.cTa

(Tan et al., 2012)

is ameliorated by DENR^GD17852

(Green et al., 2022)

is ameliorated by DENR^KK109336

(Green et al., 2022)

is exacerbated by Hrb87F^JF01757

(He et al., 2014)

is exacerbated by Hrb98DE^JF01249

(He et al., 2014)

is exacerbated by Hsap\FUS^UAS.Tag:HA

(He et al., 2014)

is ameliorated by Hsap\HNRNPA2B1^UAS.Tag:MYC

(He et al., 2014)

is exacerbated by Hsap\TARDBP^{Δ321-366.UAS.Tag:HA}

(He et al., 2014)

is ameliorated by Hsap\TARDBP^{FFLL.UAS.Tag:HA}

(He et al., 2014)

is exacerbated by Hsap\TARDBP^{G294A.UAs.Tag:MYC}

(He et al., 2014)

is exacerbated by Hsap\TARDBP^{M337V.UAS.Tag:MYC}

(He et al., 2014)

is exacerbated by Hsap\TARDBP^{NLSmut.UAS.Tag:MYC}

(He et al., 2014)

is ameliorated by Hsap\TARDBP^UAS.Tag:MYC

(He et al., 2014)

is ameliorated by Hsap\TARDBP^{UAS.cHa.Tag:HA}

(He et al., 2014)

model of primary ovarian insufficiency

is ameliorated by Pdha1^HMC04032

(Trevino et al., 2021)

is ameliorated by Sumo^HMS01540

(Trevino et al., 2021)

is ameliorated by dbe^HMS04474

(Trevino et al., 2021)

Zzzz\CGG^60.UAS.EGFP

model of fragile X-associated tremor/ataxia syndrome

is ameliorated by Hrb87F^UAS.cSa

(Tan et al., 2012)

Zzzz\CGG^103.UAS.EGFP

model of fragile X-associated tremor/ataxia syndrome

is exacerbated by kra^JF02556

(Singh et al., 2021)

is ameliorated by kra^UAS.Tag:FLAG

(Singh et al., 2021)

is exacerbated by kra^j9B6

(Singh et al., 2021)

Zzzz\CTG

Models Based on Experimental Evidence ( 8 )

Allele

Disease

Evidence

References

Zzzz\CTG^{86.UAS.Tag:MYC,Tag:FLAG}

model of neurodegenerative disease

CEA

(van Eyk et al., 2011)

Zzzz\CTG^{84.UAS.Tag:MYC,Tag:FLAG}

model of neurodegenerative disease

CEA

(van Eyk et al., 2011)

Zzzz\CTG^{term.CTG.99.UAS}

model of neurodegenerative disease

CEA with Mef2^GD5039

(van Eyk et al., 2011)

CEA with mod(mdg4)^GD16547

(van Eyk et al., 2011)

DOES NOT model neurodegenerative disease

CEA

(van Eyk et al., 2011)

Zzzz\CTG^i480.UAS.cGa

model of myotonic dystrophy type 1

CEA

(García-Puga et al., 2024, Zhang et al., 2018, Cerro-Herreros et al., 2016, Luu et al., 2016, Bargiela et al., 2015, Nguyen et al., 2015, Wong et al., 2014, García-López et al., 2011, Garcia-Lopez et al., 2008)

model of neurodegenerative disease

CEA

(Peng et al., 2021)

Zzzz\CTG^i960.UAS

model of myotonic dystrophy type 1

CEA

(Souidi et al., 2023, Picchio et al., 2018, Picchio et al., 2013)

Zzzz\CTG^i480.UAS

model of myotonic disease

CEA

(de Haro et al., 2006)

Zzzz\CTG^250.UAS.cCa

model of myotonic dystrophy type 1

CEA

(Chakraborty et al., 2018, Sellier et al., 2018, Cerro-Herreros et al., 2017, Chakraborty et al., 2015)

Zzzz\CTG^{rCUG.99.UAS.Tag:MYC,Tag:FLAG}

model of neurodegenerative disease

CEA with Zzzz\CAG^{99.UAS.Tag:MYC,Tag:FLAG}

(Samaraweera et al., 2013)

CEA

(Eyk et al., 2019)

Modifiers Based on Experimental Evidence ( 7 )

Allele

Disease

Interaction

References

Zzzz\CTG^{86.UAS.Tag:MYC,Tag:FLAG}

model of neurodegenerative disease

is exacerbated by mod(mdg4)^GD16547

(van Eyk et al., 2011)

is exacerbated by Mef2^GD5039

(van Eyk et al., 2011)

Zzzz\CTG^{84.UAS.Tag:MYC,Tag:FLAG}

model of neurodegenerative disease

is exacerbated by sgg^UAS.cBa

(van Eyk et al., 2011)

is ameliorated by sgg^KK108994

(van Eyk et al., 2011)

Zzzz\CTG^{term.CTG.99.UAS}

model of neurodegenerative disease

is exacerbated by sgg^UAS.cBa

(van Eyk et al., 2011)

Zzzz\CTG^i480.UAS.cGa

model of myotonic dystrophy type 1

is ameliorated by Diap1^UAS.cUa

(Bargiela et al., 2015)

is ameliorated by Atg8a^VDRC.cUa

(Bargiela et al., 2015)

is ameliorated by mTor^UAS.cUa

(Bargiela et al., 2015)

is ameliorated by Atg4a^KK108570

(Bargiela et al., 2015)

is exacerbated by Diap1^UAS.cUa

(Bargiela et al., 2015)

is ameliorated by Atg7^GD11671

(Bargiela et al., 2015)

is ameliorated by mbl^C.UAS

(Bargiela et al., 2015)

is ameliorated by Atg9^GD2029

(Bargiela et al., 2015)

is ameliorated by mir-277^{sponge.V2.UAS.mCherry}

(Cerro-Herreros et al., 2016)

is ameliorated by mir-304^{sponge.V2.UAS.mCherry}

(Cerro-Herreros et al., 2016)

is ameliorated by Zzzz\ABP1^ABP1c.UAS

(García-López et al., 2011)

is ameliorated by Zzzz\ABP1^ABP1r.UAS

(García-López et al., 2011)

is ameliorated by TBPH^NIG.10327R

(Llamusi et al., 2013)

Zzzz\CTG^i480.UAS

model of myotonic disease

is ameliorated by Hsap\MBNL1^{UAS.Tag:Xpress}

(de Haro et al., 2006)

model of myotonic dystrophy type 1

is ameliorated by smg^EP3556

(de Haro et al., 2013)

is exacerbated by smg¹

(de Haro et al., 2013)

Zzzz\CTG^250.UAS.cCa

model of myotonic dystrophy type 1

is ameliorated by Hsap\MBNL1^UAS.cGCa

(Sellier et al., 2018)

Zzzz\CTG^{rCUG.99.UAS.Tag:MYC,Tag:FLAG}

model of neurodegenerative disease

is ameliorated by CPV\CrPV-1A^{UASp.Tag:FLAG}

(Eyk et al., 2019)

is ameliorated by Hsap\ADAR^UAS.p110.cHa

(Eyk et al., 2019)

is ameliorated by Hsap\ADAR^UAS.p150.cHa

(Eyk et al., 2019)

Zzzz\UGGAA

Models Based on Experimental Evidence ( 2 )

Allele

Disease

Evidence

References

Zzzz\UGGAA^S.UAS

model of spinocerebellar ataxia type 31

CEA

(Ishiguro et al., 2017)

Zzzz\UGGAA^W.UAS

model of spinocerebellar ataxia type 31

CEA

(Ishiguro et al., 2017)

Modifiers Based on Experimental Evidence ( 3 )

Allele

Disease

Interaction

References

Zzzz\UGGAA^22.UAS

ameliorates amyotrophic lateral sclerosis

modeled by Hsap\TARDBP^{G298S.UAS.cIb}

(Ishiguro et al., 2017)

modeled by Hsap\TARDBP^UAS.cIb

(Ishiguro et al., 2017)

modeled by Hsap\HNRNPA2B1^D290V.UAS

(Ishiguro et al., 2017)

modeled by Hsap\FUS^UAS.cIa

(Ishiguro et al., 2017)

Zzzz\UGGAA^S.UAS

model of spinocerebellar ataxia type 31

is ameliorated by Hsap\TARDBP^ΔN.UAS

(Ishiguro et al., 2017)

is NOT ameliorated by Hsap\TARDBP^RRM.UAS

(Ishiguro et al., 2017)

is ameliorated by Hsap\FUS^UAS.cIa

(Ishiguro et al., 2017)

is NOT ameliorated by caz^KK107486

(Ishiguro et al., 2017)

is ameliorated by Hsap\TARDBP^UAS.cIb

(Ishiguro et al., 2017)

is ameliorated by Hsap\TARDBP^ΔC.UAS

(Ishiguro et al., 2017)

is ameliorated by Hsap\HNRNPA2B1^UAS.cKa

(Ishiguro et al., 2017)

is NOT ameliorated by Hrb87F^KK108162

(Ishiguro et al., 2017)

is NOT ameliorated by Hrb98DE^GD14939

(Ishiguro et al., 2017)

Zzzz\UGGAA^W.UAS

model of spinocerebellar ataxia type 31

is ameliorated by Hsap\TARDBP^UAS.cIb

(Ishiguro et al., 2017)

is ameliorated by Hsap\TARDBP^ΔN.UAS

(Ishiguro et al., 2017)

is exacerbated by TBPH^GD6943

(Ishiguro et al., 2017)

is ameliorated by Hsap\HNRNPA2B1^UAS.cKa

(Ishiguro et al., 2017)

is exacerbated by nonA^GD11253

(Ishiguro et al., 2017)

is exacerbated by mle^GD1488

(Ishiguro et al., 2017)

is NOT ameliorated by Hrb98DE^GD14939

(Ishiguro et al., 2017)

is ameliorated by Hsap\TARDBP^ΔC.UAS

(Ishiguro et al., 2017)

is NOT ameliorated by Hsap\TARDBP^RRM.UAS

(Ishiguro et al., 2017)

is ameliorated by Hsap\FUS^UAS.cIa

(Ishiguro et al., 2017)

is NOT ameliorated by caz^KK107486

(Ishiguro et al., 2017)

is exacerbated by DnaJ-1^UAS.cIa

(Ishiguro et al., 2017)

is NOT ameliorated by mahe^GD17235

(Ishiguro et al., 2017)

is NOT ameliorated by Hrb87F^KK108162

(Ishiguro et al., 2017)

ameliorates amyotrophic lateral sclerosis

modeled by Hsap\TARDBP^UAS.cIb

(Ishiguro et al., 2017)

Zzzz\CCTG

Models Based on Experimental Evidence ( 5 )

Allele

Disease

Evidence

References

Zzzz\CCTG^UAS.475

model of myotonic dystrophy type 2

CEA

(Yu et al., 2015)

Zzzz\CCTG^UAS.525

model of myotonic dystrophy type 2

CEA

(Yu et al., 2015)

Zzzz\CCTG^UAS.700

model of myotonic dystrophy type 2

CEA

(Yu et al., 2015)

Zzzz\CCTG^1100.UAS

model of myotonic dystrophy type 2

CEA

(Sellier et al., 2018, Cerro-Herreros et al., 2017)

Zzzz\CCTG^UAS.106

model of myotonic dystrophy type 2

CEA

(Yenigun et al., 2017)

Modifiers Based on Experimental Evidence ( 3 )

Allele

Disease

Interaction

References

Zzzz\CCTG^UAS.700

model of myotonic dystrophy type 2

is ameliorated by Hsap\MBNL1^35.UAS

(Yu et al., 2015)

is ameliorated by Hsap\MBNL1^UAS.cGCa

(Yu et al., 2015)

Zzzz\CCTG^1100.UAS

model of myotonic dystrophy type 2

is ameliorated by Hsap\MBNL1^UAS.cGCa

(Sellier et al., 2018)

is ameliorated by Hsap\RBFOX1^UAS.EGFP

(Sellier et al., 2018)

Zzzz\CCTG^UAS.106

model of myotonic dystrophy type 2

is ameliorated by BacA\p35^UAS.cUa

(Yenigun et al., 2017)

is ameliorated by Hsap\MBNL1^UAS.cYa

(Yenigun et al., 2017)

is exacerbated by mbl^KG08885

(Yenigun et al., 2017)

Alleles Representing Disease-Implicated Variants

Genetic Tools, Stocks and Reagents

Sources of Stocks

Contact lab of origin for a reagent not available from a public stock center.

Bloomington Stock Center Disease Page

Related mammalian, viral, bacterial, or synthetic transgenes

Allele

Transgene

Publicly Available Stocks

Zzzz\CTG^{50.UAS.Tag:MYC,Tag:FLAG}

P{UAS-CTG.50}

Zzzz\CTG^{rCUG.99.UAS.Tag:MYC,Tag:FLAG}

P{UAS-rCUG.100}

Zzzz\CTG^{86.UAS.Tag:MYC,Tag:FLAG}

P{UAS-CTG.86}

Zzzz\CTG^{84.UAS.Tag:MYC,Tag:FLAG}

P{UAS-CTG.84}

Zzzz\CTG^{term.CTG.99.UAS}

Zzzz\CTG^i480.UAS.cGa

P{UAS-CTG.i480.G}

Zzzz\CTG^{84.UAS.cvEa.Tag:MYC,Tag:FLAG}

P{UAS-CTG.84.vE.MYC.FLAG}

Zzzz\CTG^i240.UAS

P{UAS-iCTG.240}

Zzzz\CTG^i480.UAS.cPa

Zzzz\CTG^16.UASp.lacZ

P{UASp-CTG.16}

Zzzz\CTG^{19.UAS.DsRed2}

P{UAS-DsRed-CTG19}

w¹¹¹⁸; P{UAS-DsRed-CTG19}1.1

Zzzz\CTG^{130.UAS.DsRed2}

P{UAS-DsRed-CTG130}

w¹¹¹⁸; P{UAS-DsRed-CTG130}1.3/TM6B, Tb¹

Zzzz\CTG^{200.UAS.DsRed2}

P{UAS-DsRed-CTG200}

w¹¹¹⁸; P{UAS-DsRed-CTG200}11.1

Zzzz\CTG^{230.UAS.DsRed2}

P{UAS-DsRed-CTG230}

w¹¹¹⁸; P{UAS-DsRed-CTG230}10.1/TM6B, Tb⁺

Zzzz\CTG^{250.UAS.DsRed2}

P{UAS-DsRed-CTG250}

w¹¹¹⁸; P{UAS-DsRed-CTG250}3.2/TM6B, Tb¹

Zzzz\CTG^{270.UAS.DsRed2}

P{UAS-DsRed-CTG270}

w¹¹¹⁸; P{UAS-DsRed-CTG270}13.1

Zzzz\CCG^90.UAS.EGFP

P{UAS-CCG90-EGFP}

Zzzz\CGG^103.UAS.EGFP

Zzzz\CGG^{100.5'UTR.UAS.GFP}

P{UAS-CGG100-5'UTR-GFP}

Zzzz\CGG^{100.5'ATG.UAS.GFP}

P{UAS-CGG100-5'ATG-GFP}

Zzzz\CGG^{100.5'Stop.UAS.GFP}

P{UAS-CGG100-5'Stop-GFP}

Zzzz\CGG^{100.3'UTR.UAS.GFP}

P{UAS-GFP-CGG100-3'UTR}

Zzzz\GGGGCC^{8A.UAS.DsRed2}

M{UAS-DsRed2-(G4C2)8A}

Zzzz\GGGGCC^{32.UAS.DsRed2}

M{UAS-DsRed2-(G4C2)32}

Zzzz\GGGGCC^{64.UAS.DsRed2}

M{UAS-DsRed2-(G4C2)64}

Zzzz\GGGGCC^{8B.UAS.DsRed2}

M{UAS-DsRed2-(G4C2)8B}

Zzzz\GGGGCC^{56.UAS.DsRed2}

M{UAS-DsRed2-(G4C2)56}

Zzzz\GGGGCC^{38.UAS.DsRed2}

M{UAS-DsRed2-(G4C2)38}

Zzzz\GGGGCC^{12.LDS.UAS.GR-GFP}

P{UAS-LDS-(G4C2)12.GR-GFP}

P{UAS-LDS-(G4C2)12.GR-GFP}2, w¹¹¹⁸

Zzzz\GGGGCC^{44.LDS.UAS.GR-GFP}

P{UAS-LDS-(G4C2)44.GR-GFP}

w¹¹¹⁸; P{UAS-LDS-(G4C2)44.GR-GFP}9

w¹¹¹⁸; P{UAS-(GGGGCC)₂₉}48-12/SM6b

Zzzz\GGGGCC^49.UAS

P{UAS-(GGGGCC)₄₉}

w¹¹¹⁸; P{UAS-(GGGGCC)₄₉}48-3.1/TM6C, Sb¹

Zzzz\GGGGCC^8.UAS

P{UAS-(GGGGCC)₈}

w¹¹¹⁸; P{UAS-(GGGGCC)₈}48-2/TM6C, Sb¹

Zzzz\GGGGCC^{44.UAS.LDS.GFP}

P{UAS-LDS.(GGGGCC)₄₄.GR-GFP}

Zzzz\GGGGCC^GFP-iC3.UAS

P{UAS-GFP-iC3}

Zzzz\GGGGCC^{GFP-iC242.UAS}

P{UAS-GFP-iC242}

Zzzz\GGGGCC^{GFP-iC484.UAS}

P{UAS-GFP-iC484}

Zzzz\GGGGCC^{0+G4C2.UAS.EGFP}

P{UAS-0+(G4C2)-EGFP}

Zzzz\GGGGCC^{1+G4C2.UAS.EGFP}

P{UAS-1+(G4C2)-EGFP}

Zzzz\GGGGCC^{2+G4C2.UAS.EGFP}

P{UAS-2+(G4C2)-EGFP}

Zzzz\GGGGCC^69.CT.UAS

P{UAS-GGGGCC.69.CT}

Zzzz\GGGGCC^69.ΔCT.UAS

P{UAS-GGGGCC.69.DeltaCT}

Zzzz\GGGGCC^{1+G4C2x28.UAS.EGFP}

P{UAS-1+(G4C2x28)-EGFP}

Zzzz\GGGGCC^107.AS.UAS

P{UAS-GGGGCC.107.AS}

Zzzz\GGGGCC^EGFP.i-106RO

P{UAS-EGFP.i-106RO}

Zzzz\GGGGCC^{EGFP.i-108RO.AS}

P{UAS-EGFP.i-108RO.AS}

Zzzz\GGGGCC^800RO.UAS

P{UAS-GGGGCC.800RO}

Zzzz\GGGGCC^1000RO.UAS

P{UAS-GGGGCC.1000RO}

Zzzz\GGGGCC^{2000-5000RO.UAS}

P{UAS-GGGGCC.2000-5000RO}

Zzzz\GGGGCC^3.UAS.EGFP

P{UAS-(GGGGCC)₃-EGFP}

Zzzz\GGGGCC^30.UAS.EGFP

P{UAS-(GGGGCC)₃₀-EGFP}

Zzzz\GGGGCC^RO-36.UAS

P{UAS-GGGGCC.36-RO}

w¹¹¹⁸; P{UAS-GGGGCC.36-RO}attP40

Zzzz\GGGGCC^RO-108.UAS

P{UAS-GGGGCC.108-RO}

w¹¹¹⁸; P{UAS-GGGGCC.108-RO}attP40

Zzzz\GGGGCC^RO-288.UAS

P{UAS-GGGGCC.288-RO}

w¹¹¹⁸; P{UAS-GGGGCC.288-RO}attP40

Zzzz\GGGGCC^3.UAS

P{UAS-poly-GP.3}

w¹¹¹⁸; P{UAS-GGGGCC.3}attP40

Zzzz\GGGGCC^36.UAS

P{UAS-poly-GP.36}

w¹¹¹⁸; P{UAS-GGGGCC.36}attP40

Zzzz\GGGGCC^103.UAS

P{UAS-GGGGCC.103}

Zzzz\GGGGCC^8.UAS.EGFP

P{UAS-GGGGCC.8}

Zzzz\GGGGCC^28.UAS.EGFP

P{UAS-GGGGCC.28}

Zzzz\GGGGCC^58.UAS.EGFP

w¹¹¹⁸; P{UAS-CCTG.16}41

Zzzz\CCTG^UAS.200

P{UAS-CCTG.200}

w¹¹¹⁸; P{UAS-CCTG.200}26.1

Zzzz\CCTG^UAS.475

P{UAS-CCTG.475}

w¹¹¹⁸; P{UAS-CCTG.475}1.3

Zzzz\CCTG^UAS.525

P{UAS-CCTG.525}

w¹¹¹⁸; P{UAS-CCTG.525}30.2

w¹¹¹⁸; P{UAS-CCTG.720}9.1

Selected Drosophila transgenes

Allele

Transgene

Publicly Available Stocks

RNAi constructs available

Allele

Transgene

Publicly Available Stocks

Selected Drosophila classical alleles

Allele

Allele class

Mutagen

Publicly Available Stocks

References (74)

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