FlyBase Gene Report: Zzzz\poly-PR

(Rossi et al., 2023, Xu et al., 2023, Farrugia et al., 2022, Fumagalli et al., 2021, Kim et al., 2021, Park et al., 2021, Marmor-Kollet et al., 2020, Park et al., 2020, Ramesh et al., 2020, Xu and Xu, 2018)

CEC

(Kim et al., 2019, Xu et al., 2019)

Zzzz\poly-PR^PO-100.UAS

model of C9orf72 frontotemporal dementia and/or amyotrophic lateral sclerosis

CEA

(Morón-Oset et al., 2023, Farrugia et al., 2022, Fumagalli et al., 2021, Park et al., 2020)

CEC

(Lu et al., 2019)

model of frontotemporal dementia

CEA

(Mizielinska et al., 2014)

model of amyotrophic lateral sclerosis

CEA

(Mizielinska et al., 2014)

Zzzz\poly-PR^{80.UAS.Tag:FLAG}

model of C9orf72 frontotemporal dementia and/or amyotrophic lateral sclerosis

CEA

(Li et al., 2020, Yang et al., 2015)

Zzzz\poly-PR^36.UAS.EGFP

model of C9orf72 frontotemporal dementia and/or amyotrophic lateral sclerosis

CEA

(Xu and Xu, 2018)

Zzzz\poly-PR^{25.UAS.Tag:FLAG}

model of C9orf72 frontotemporal dementia and/or amyotrophic lateral sclerosis

CEA

(Boeynaems et al., 2016)

Zzzz\poly-PR^{UAS.Tag:GS-TAP}

model of C9orf72 frontotemporal dementia and/or amyotrophic lateral sclerosis

CEA

(Moens et al., 2019)

Zzzz\poly-PR^{36.UAS.mCherry}

model of C9orf72 frontotemporal dementia and/or amyotrophic lateral sclerosis

CEA

(Morón-Oset et al., 2023)

Zzzz\poly-PR^{100.UAS.mCherry}

model of C9orf72 frontotemporal dementia and/or amyotrophic lateral sclerosis

CEA

(Morón-Oset et al., 2023)

Zzzz\poly-PR^{1100.UAS.EGFP}

model of C9orf72 frontotemporal dementia and/or amyotrophic lateral sclerosis

CEA

(Webster et al., 2025, West et al., 2020)

Potential Models Based on Orthology ( 0 )

Human Ortholog

Disease

Evidence

References

Modifiers Based on Experimental Evidence ( 5 )

Allele

Disease

Interaction

References

Zzzz\poly-PR^PO-36.UAS

model of amyotrophic lateral sclerosis

is NOT ameliorated by SF2^GD12097

(Hautbergue et al., 2017)

model of C9orf72 frontotemporal dementia and/or amyotrophic lateral sclerosis

is ameliorated by lwr^UAS.cAa

(Marmor-Kollet et al., 2020)

is ameliorated by Upf1^UASp.GFP

(Xu et al., 2019)

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 exacerbated by CG11414^HMC06381

(Park et al., 2021)

is exacerbated by CG11414^HMJ23120

(Park et al., 2021)

is exacerbated by CG14207^NIG.14207R

(Park et al., 2021)

is exacerbated by Capr^NIG.18811R

(Park et al., 2021)

is exacerbated by Clbn^GD7403

(Park et al., 2021)

is exacerbated by Edc3^NIG.6311R

(Park et al., 2021)

is exacerbated by Rack1^GL00637

(Park et al., 2021)

is exacerbated by Rack1^NIG.7111R

(Park et al., 2021)

is exacerbated by PAN3^NIG.11486R

(Park et al., 2021)

is exacerbated by PpV^NIG.12217R

(Park et al., 2021)

is exacerbated by Smg6^NIG.6369R

(Park et al., 2021)

is exacerbated by TER94^JF03402

(Park et al., 2021)

is exacerbated by Upf2^NIG.2253R

(Park et al., 2021)

is exacerbated by Upf3^NIG.11184R

(Park et al., 2021)

is exacerbated by Usp30^NIG.3016R

(Park et al., 2021)

is exacerbated by cup^NIG.11181R

(Park et al., 2021)

is exacerbated by eIF2β^NIG.4153R

(Park et al., 2021)

is exacerbated by eRF1^NIG.5605R

(Park et al., 2021)

is exacerbated by pico^NIG.11940R

(Park et al., 2021)

is exacerbated by pix^JF01190

(Park et al., 2021)

is exacerbated by rig^NIG.11171R

(Park et al., 2021)

is exacerbated by tral^NIG.10686R

(Park et al., 2021)

is exacerbated by stau^UAS.GFP

(Kim et al., 2021)

is exacerbated by Fib^KK108001

(Kim et al., 2021)

is exacerbated by stau^{UAS.Tag:NLS(Unk),Tag:HA}

(Kim et al., 2021)

is ameliorated by TBPH^UAS.cUa

(Kim et al., 2021)

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

(Xu et al., 2023)

is exacerbated by ArfGAP1^HMJ30212

(Rossi et al., 2023)

is exacerbated by ergic53^HMC03809

(Rossi et al., 2023)

Zzzz\poly-PR^PO-100.UAS

exacerbates C9orf72 frontotemporal dementia and/or amyotrophic lateral sclerosis

modeled by Zzzz\poly-GA^200.UAS

(Morón-Oset et al., 2023)

model of C9orf72 frontotemporal dementia and/or amyotrophic lateral sclerosis

is exacerbated by Set8^GD10930

(Lu et al., 2019)

is ameliorated by Su(var)3-3^HMS00608

(Lu et al., 2019)

is ameliorated by l(3)mbt^HMS01466

(Lu et al., 2019)

is exacerbated by p53^GL01220

(Lu et al., 2019)

is ameliorated by SERCA^KK107371

(Park et al., 2020)

Zzzz\poly-PR^{80.UAS.Tag:FLAG}

model of C9orf72 frontotemporal dementia and/or amyotrophic lateral sclerosis

is ameliorated by Akt^UAS.Exel

(Li et al., 2020)

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

(Li et al., 2020)

is ameliorated by TER94^UAS.cRa

(Li et al., 2020)

Zzzz\poly-PR^{25.UAS.Tag:FLAG}

model of C9orf72 frontotemporal dementia and/or amyotrophic lateral sclerosis

is ameliorated by Rcc1^KK100628

(Boeynaems et al., 2016)

is ameliorated by Tnpo^KK108990

(Boeynaems et al., 2016)

is ameliorated by Apl^KK115697

(Boeynaems et al., 2016)

is ameliorated by Art1^JF01306

(Boeynaems et al., 2016)

is ameliorated by emb^JF01311

(Boeynaems et al., 2016)

is ameliorated by Art6^GL01070

(Boeynaems et al., 2016)

is ameliorated by Art7^GL01071

(Boeynaems et al., 2016)

is ameliorated by FBXO11^KK100670

(Boeynaems et al., 2016)

is ameliorated by Fs(2)Ket^KK100714

(Boeynaems et al., 2016)

is ameliorated by Impβ11^KK105693

(Boeynaems et al., 2016)

is ameliorated by Kap-α3^KK101268

(Boeynaems et al., 2016)

is exacerbated by Mgtor^KK102690

(Boeynaems et al., 2016)

is ameliorated by Nup107^KK108047

(Boeynaems et al., 2016)

is ameliorated by Nup154^HMS01189

(Boeynaems et al., 2016)

is exacerbated by Nup44A^HMS00736

(Boeynaems et al., 2016)

is ameliorated by Nup50^HMS01054

(Boeynaems et al., 2016)

is exacerbated by Nup62^GLV21060

(Boeynaems et al., 2016)

is exacerbated by Nup93-1^JF01712

(Boeynaems et al., 2016)

is ameliorated by RanGAP^KK102830

(Boeynaems et al., 2016)

Zzzz\poly-PR^{1100.UAS.EGFP}

exacerbates C9orf72 frontotemporal dementia and/or amyotrophic lateral sclerosis

modeled by Zzzz\poly-GR^{1136.UAS.EGFP}

(West et al., 2020)

model of C9orf72 frontotemporal dementia and/or amyotrophic lateral sclerosis

is exacerbated by Zzzz\poly-GR^{1136.UAS.EGFP}

(West et al., 2020)

is ameliorated by rept^UAS.ORF

(Webster et al., 2025)

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

(Webster et al., 2025)

Disease Associations of Human Orthologs (via DIOPT v9.1 and OMIM)

Note that ortholog calls supported by only 1 or 2 algorithms (DIOPT score < 3) are not shown.

Homo sapiens (Human)

Gene name

Score

OMIM

OMIM Phenotype

DO term

Complementation?

Transgene?

Functional Complementation Data

Functional complementation data is computed by FlyBase using a combination of the orthology data obtained from DIOPT and OrthoDB and the allele-level genetic interaction data curated from the literature.

Interactions

Summary of Physical Interactions

Interaction Browsers

Summary of Genetic Interactions

Interaction Browsers

Starting gene(s)

Interaction type

Interacting gene(s)

Reference

Starting gene(s)

Interaction type

Interacting gene(s)

Reference

External Data

Linkouts

Class of Gene

engineered_foreign_region

(FlyBase Curators, 1992-)

Stocks and Reagents

Stocks (2)

Bloomington

58694

w¹¹¹⁸; P{UAS-poly-PR.PO-36}attP40

58698

w¹¹¹⁸; P{UAS-poly-PR.PO-100}attP40

Other Comments

Relationship to Other Genes

Source for database merge of

Additional comments

Nomenclature History

Source for database identify of

Nomenclature comments

Etymology

Synonyms and Secondary IDs (6)

Reported As

Symbol Synonym

(Boeynaems et al., 2016)

Zzzz\poly-PR

poly-PR

(Mizielinska et al., 2014, Wen et al., 2014)

Name Synonyms

C9RAN poly-dipeptide PR

(Wen et al., 2014)

poly-(Proline-Arginine)

poly-(proline-arginine)

(Mizielinska et al., 2014)

Secondary FlyBase IDs

Datasets (0)

Study focus (0)

Experimental Role

Project

Project Type

Title

Study result (0)

Result

Result Type

Title

External Crossreferences and Linkouts ( 0 )

References (39)

Report Sections