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General Information
Symbol
Hsap\HTTNT.128Q.UAS
Species
H. sapiens
Name
FlyBase ID
FBal0197899
Feature type
allele
Associated gene
Associated Insertion(s)
Carried in Construct
Also Known As
NT-HTT128Q
Allele class
Nature of the Allele
Allele class
Mutations Mapped to the Genome
 
Type
Location
Additional Notes
References
Associated Sequence Data
DNA sequence
Protein sequence
 
 
Progenitor genotype
Carried in construct
Cytology
Nature of the lesion
Statement
Reference

UAS regulatory sequences drive expression of amino acids 1-276 of Hsap\HTT which contains an expanded 128Q repeat.

Allele components
Product class / Tool use(s)
Encoded product / tool
Expression Data
Reporter Expression
Additional Information
Statement
Reference
 
Marker for
Reflects expression of
Reporter construct used in assay
Human Disease Associations
Disease Ontology (DO) Annotations
Models Based on Experimental Evidence ( 1 )
Modifiers Based on Experimental Evidence ( 1 )
Disease
Interaction
References
is ameliorated by S-Lap1GD11904
is exacerbated by CG15445KK104296
is ameliorated by Pvf1d06222
is exacerbated by Sec61β07214
is exacerbated by Sec61γVDRC.cUa
is exacerbated by Pvf1KG01713
is ameliorated by DgkεGD1964
is exacerbated by GAPcenAf01044
is ameliorated by DopEcRMI02790
is ameliorated by DopEcRKK111211
Comments on Models/Modifiers Based on Experimental Evidence ( 0 )
 
Phenotypic Data
Phenotypic Class
Phenotype Manifest In
Detailed Description
Statement
Reference

Expression of Hsap\HTTNT.128Q.Scer\UAS under the control of Scer\GAL4elav-C155 induces rapid and steep age-progressive decline of locomotor abilities of adult flies.

Expression of Hsap\HTTNT.128Q.Scer\UAS in the nervous system (under the control of Scer\GAL4elav-C155) induces a reproducible and quantifiable motor performance impairment as measured by the percentage of Hsap\HTTNT.128Q.Scer\UAS flies able to ascend the walls of a glass cylinder, compared to controls.

Expression of Hsap\HTTNT.128Q.Scer\UAS in the developing eye, under the control of Scer\GAL4GMR.PF, results in cell loss and retinal shrinking.

Expression of Hsap\HTTNT.128Q.Scer\UAS in the nervous system, under the control of Scer\GAL4elav-C155 leads to progressive motor performance deficits compared to wild-type animals.

Expression of Hsap\HTTNT.128Q.Scer\UAS under the control of Scer\GAL4GMR.PU results in age-progressive retina degeneration with severely disorganized or missing photoreceptors.

Expression of Hsap\HDNT.128Q.Scer\UAS under the control of Scer\GAL4GMR.PF causes progressive retinal degeneration with severe vacuolisation.

External Data
Interactions
Show genetic interaction network for Enhancers & Suppressors
Phenotypic Class
Enhanced by
Suppressed by
NOT suppressed by
Phenotype Manifest In
Additional Comments
Genetic Interactions
Statement
Reference
Xenogenetic Interactions
Statement
Reference

The accelerated age-progressive decline of locomotor ability of adult flies expressing Hsap\HTTNT.128Q.Scer\UAS under the control of Scer\GAL4elav-C155 is strongly improved by co-expression of either DopEcRMI02790 or DopEcRKK111211 and further worsened by co-expression of either GAPcenAKK101516 or GAPcenAf01044.

Co-expression of Hsap\NUB1Scer\UAS.cLa in the nervous system, under the control of Scer\GAL4elav-C155 suppresses the motor performance impairments seen upon expression of Hsap\HTTNT.128Q.Scer\UAS.

Expression of Hsap\NUB1Scer\UAS.cLa in the fly retina, under the control of Scer\GAL4GMR.PF, suppresses the cell loss and retinal shrinkage caused by Hsap\HTTNT.128Q.Scer\UAS expression.

Co-expression of CG15445KK104296 with Hsap\HTTNT.128Q.Scer\UAS in the nervous system, under the control of Scer\GAL4elav-C155 enhances the motor performance defects found upon expression of Hsap\HTTNT.128Q.Scer\UAS alone.

Expression of CG15445KK104296 under the control of Scer\GAL4GMR.PF exacerbates the retinal phenotype seen upon Hsap\HTTNT.128Q.Scer\UAS expression.

Co-expression of S-Lap1GD11904 with Hsap\HTTNT.128Q.Scer\UAS in the nervous system, under the control of Scer\GAL4elav-C155 enhances the motor performance defects found upon expression of Hsap\HTTNT.128Q.Scer\UAS alone.

Co-expression of Pvf1d06222 with Hsap\HTTNT.128Q.Scer\UAS in the nervous system, under the control of Scer\GAL4elav-C155 suppresses the motor performance defects found upon expression of Hsap\HTTNT.128Q.Scer\UAS alone.

Co-expression of Pvf1KG01713 with Hsap\HTTNT.128Q.Scer\UAS in the nervous system, under the control of Scer\GAL4elav-C155 enhances the motor performance defects found upon expression of Hsap\HTTNT.128Q.Scer\UAS alone.

Co-expression of Sec61β07214 with Hsap\HTTNT.128Q.Scer\UAS in the nervous system, under the control of Scer\GAL4elav-C155 enhances the motor performance defects found upon expression of Hsap\HTTNT.128Q.Scer\UAS alone.

Co-expression of Sec61γVDRC.cUa with Hsap\HTTNT.128Q.Scer\UAS in the nervous system, under the control of Scer\GAL4elav-C155 enhances the motor performance defects found upon expression of Hsap\HTTNT.128Q.Scer\UAS alone.

Decreasing the level of Dgkε through expression of DgkεGD1964 under the control of Scer\GAL4elav-C155 significantly ameliorates the toxic effects of Hsap\HTTNT.128Q.Scer\UAS expression.

Neither the age-progressive retina degeneration nor the decline of climbing abilities characteristic for adult flies expressing Hsap\HTTNT.128Q.Scer\UAS under the control of the Scer\GAL4GMR.PU or the Scer\GAL4nrv2.PU driver, respectively, can be suppressed by combination with Atx2X1 in a heterozygous state.

Co-expression of Hsap\STUB1Scer\UAS.cAa suppresses the retina defects caused by expression of Hsap\HDNT.128Q.Scer\UAS under the control of Scer\GAL4GMR.PF.

Complementation and Rescue Data
Comments
Images (0)
Mutant
Wild-type
Stocks (0)
Notes on Origin
Discoverer
External Crossreferences and Linkouts ( 0 )
Synonyms and Secondary IDs (3)
Reported As
Symbol Synonym
Hsap\HDNT.128Q.Scer\UAS
Hsap\HTTNT.128Q.Scer\UAS
Hsap\HTTNT.128Q.UAS
Name Synonyms
Secondary FlyBase IDs
    References (7)