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General Information
Symbol
Hsap\ATXN182Q.UAS
Species
H. sapiens
Name
FlyBase ID
FBal0119474
Feature type
allele
Associated gene
Associated Insertion(s)
Carried in Construct
Also Known As
UAS-SCA182Q, SCA182Q, UAS-Atxn1-82Q, ATXN1(82Q), UAS-SCA182Q
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
A Hsap\ATXN1 cDNA containing 82 CAG repeats is driven by UASt sequences.
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 exacerbated by NmnatUAS.PC
is ameliorated by NmnatUAS.PD
is ameliorated by Akt104226
is ameliorated by WnkKK102654
is ameliorated by AdukKK102129
is ameliorated by SdrKK104654
is ameliorated by BubR1DN.UAS
is ameliorated by JIL-1KK101300
is ameliorated by JIL-1KG08229
is ameliorated by Dsor1G79
is ameliorated by licKK108550
is ameliorated by FntaKK100303
is ameliorated by rl1
is ameliorated by drkKG03077
is ameliorated by SosKG10074
is ameliorated by Ras85D06677
is ameliorated by Ras64BGD1517
is ameliorated by RafKK100982
is ameliorated by Spf45J23
is ameliorated by DnaJ-1EP411
is exacerbated by Sin3AdQ4
is exacerbated by skdL7062
is exacerbated by GstS106253
is ameliorated by vibEP513
is exacerbated by eff8
is exacerbated by taraEP3463
is exacerbated by cpoEP3378
is exacerbated by Sirt1EP2300
is exacerbated by pumEP3461
is ameliorated by pumbem
is ameliorated by 14-3-3εex4
is ameliorated by ps3
is exacerbated by HDAC104556
is exacerbated by CtBP03463
is exacerbated by Diap14
is exacerbated by Sc205634
is ameliorated by psj449
is ameliorated by Akt104226
is ameliorated by mubEP3623
is exacerbated by mub04093
is ameliorated by vibEP651
is exacerbated by sovKG00226
is ameliorated by sovKG00226
is exacerbated by Hsap\NLKUAS.cJa
is ameliorated by nmoadk2
Comments on Models/Modifiers Based on Experimental Evidence ( 0 )
 
Phenotypic Data
Phenotypic Class
Phenotype Manifest In
interneuron & cell body, with Scer\GAL4VNC.ap
Detailed Description
Statement
Reference
Expression of Hsap\ATXN182Q.Scer\UAS driven by Scer\GAL4nrv2.PS induces an age-dependent (significant at day 10 and 20 but not day 1) decline in climbing performance and signs of apoptosis in the adult brain (2 day old female).
Increased DNA damage is seen in the motor neurons of flies expressing Hsap\ATXN182Q.Scer\UAS under the control of Scer\GAL4RapGAP1-OK6 compared to controls. A shortened lifespan is observed when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6. Feeding flies with a specific inhibitor of grp (CHIR-24) suppresses this phenotype in a concentrations-dependent manner.
Expression of Hsap\ATXN182Q.Scer\UAS under the control of Scer\GAL4GMR.PU results in reduced retina thickness compared to controls.
Expression of Hsap\ATXN182Q.Scer\UAS in the eye under the control of Scer\GAL4GMR.PU results in retinal depigmentation, disruption of the structured eye pattern and retinal degeneration. The eye is yellow-colored and occasionally shows necrotic patches on the external surface.
Expression of Hsap\ATXN182Q.Scer\UAS under the control of Scer\GAL4GMR.PF induces an external eye phenotype and photoreceptor degeneration compared to controls. Expression of Hsap\ATXN182Q.Scer\UAS under the control of Scer\GAL4nrv2.PS leads to a decreased in adult lifespan compared to controls.
Larval ddaE neurons expressing Hsap\ATXN182Q.UAS under the control of Scer\GAL4221 show a significant increase in growing microtubules within dendrites, as compared to neurons in controls.
Expression of Hsap\ATXN182Q.Scer\UAS under the control of Scer\GAL4GMR.PU results in a rough eye phenotype.
Expression of Hsap\ATXN182Q.Scer\UAS under the control of Scer\GAL4elav.Switch.PO during the adult stage (using RU486) results in a reduction in mean lifespan compared to controls.
Larvae expressing Hsap\ATXN182Q.Scer\UAS in class IV da neurons under the control of Scer\GAL4ppk.PG exhibit striking dendritic abnormalities compared to controls. Terminal dendritic branching and complexity is greatly reduced compared to controls, whereas the major dendrite branches are unaffected. Expression of Hsap\ATXN182Q.Scer\UAS in dorsal cluster neurons under the control of Scer\GAL4109(2)80 results in a reduction in F-actin structures in distal dendrites.
Expression of Hsap\ATX182Q.Scer\UAS using Scer\GAL4GMR.PF results in depigmented eyes with moderate roughening; ommatidial arrays are intact although a little disorganized. Expression of Hsap\ATX182Q.Scer\UAS using Scer\GAL4elav-C155 results in death of 48% of progeny as early embryos and the rest before adult emergence.
Some (~50%) of Scer\GAL4gcm.PU Hsap\ATXN182Q.Scer\UAS pupae have remarkably small heads. Head size is not significantly affected in Scer\GAL4repo Hsap\ATXN182Q.Scer\UAS pupae. In mild cases, Scer\GAL4gcm.PU Hsap\ATXN182Q.Scer\UAS pupal brains show a relatively normal gross structure, whereas the size of each brain structure is 50-60% of normal length. Vacuolations are observed in some parts. In severe cases the brain structure is severely destroyed and the relationship to surrounding head tissues is distorted. The gross structure of the brain is relatively preserved in Scer\GAL4repo Hsap\ATXN182Q.Scer\UAS pupae. However, neuropils are coarse and the number of neurons, especially photoreceptor neurons in the retina, is reduced. The number and density of neurons surrounding optic lobes is remarkably decreased, the number of axons from photoreceptor neurons is reduced, and the axonal pathway from retina to lamina and lamina to medulla is remarkably distorted in Scer\GAL4gcm.PU Hsap\ATXN182Q.Scer\UAS and especially in Scer\GAL4repo Hsap\ATXN182Q.Scer\UAS pupal brains.
Expression of Hsap\ATXN182Q.Scer\UAS under the control of Scer\GAL4GMR.PU causes an external eye phenotype consisting of ommatidial disorganisation and interommatididal bristle loss.The flies exhibit a retinal phenotype, characterized by a decrease in the length of the retina, tissue loss and shortened and abnormal photoreceptor neurons. Flies expressing Hsap\ATXN182Q.Scer\UAS under the control of Scer\GAL4nrv2.PS exhibit progressive climbing defects.
Flies expressing Hsap\ATXN182Q.Scer\UAS under the control of Scer\GAL4GMR.PF at 30[o]C exhibit retinal degeneration, ommatidial disorganisation and fusion and interommatidial bristle loss. A less severe phenotype is seen at 25[o]C.
Expression of Scer\GAL4GMR.PF>Hsap\ATXN182Q.Scer\UAS leads to rough eye and reduced rhabdomere size.
Expression of Hsap\ATXN182Q.Scer\UAS under the control of Scer\GAL4GMR.PU results in disorganized ommatidia and interommatidial bristles, tissue loss in the retina as well as morphological defects in the photoreceptor neurons of adult eye. Expression under the Scer\GAL4nrv2.PU driver leads to age-progressive impairment of the locomotor performance (in a climbing assay) and shorter lifespan of the adult flies compared to controls. Expression under the control of Scer\GAL4sca-109-68 leads to a mild loss of macrochaete in the adult thorax.
Flies expressing Hsap\ATX182Q.Scer\UAS under the control of Scer\GAL4GMR.PF have a severe external eye phenotype with disorganised and fused ommatidia and loss of interommatidial bristles. There is severe retinal degeneration, as evident by reduction in depth of the retina.
Expression of Hsap\ATX182Q.Scer\UAS in the eye, under the control of Scer\GAL4GMR.PF, causes ommatidial disorganization and fusion, as well as interommatidial bristle loss. Expression of Hsap\ATX182Q.Scer\UAS in the wing pouch, driven by Scer\GAL4C5, results in the production of smaller wings which have a thicker L3 vein, a missing posterior crossvein, and ectopic vein formation. These phenotypes are most prominent in the posterior part of the wing, where transgene expression is highest. Males showing high levels of Hsap\ATX182Q.Scer\UAS expression, when the transgene is inserted in the X chromosome, have atrophic wings.
Overexpression of Hsap\ATX182Q.Scer\UAS, under the control of Scer\GAL4ninaE.PT results in neurodegeneration in the eye. At 2 days after eclosion, no obvious morphological changes in the photoreceptor cells are observed. At 28 days, many large holes and a loss of cell integrity is observed. On average, only one or two photoreceptor cells remain out of the eight found in normal eyes. Overexpression of Hsap\ATX182Q.Scer\UAS, under the control of Scer\GAL4GMR.PF induces eye abnormalities. The external structures of the eyes exhibit disorganised ommatidia and a loss of interommatidial bristles. They also exhibit a loss of rhabdomeres and tissue integrity. Overexpression of Hsap\ATX182Q.Scer\UAS, under the control of Scer\GAL4GMR.PF also generates extra wing-vein tissue. Overexpression of Hsap\ATX182Q.Scer\UAS, under the control of Scer\GAL4C5 in the wing pouch at 27oC results in several vein abnormalities and wing atrophy. Overexpression of Hsap\ATX182Q.Scer\UAS, under the control of Scer\GAL4C96 in the wing margin causes bristle abnormalities including patterning defects and bristle loss. Overexpression of Hsap\ATX182Q.Scer\UAS in the sensory organ precursor cells under the control of Scer\GAL4sca-109-68 causes a loss of thoracic bristles.
When Hsap\ATX182Q.Scer\UAS is driven by Scer\GAL4GMR.PF at 23oC, a mild degenerative phenotype is seen in the eye with a moderate disruption of the regular external lattice, a thinner retina layer, and mildly abnormal rhabdomeres.
External Data
Interactions
Show genetic interaction network for Enhancers & Suppressors
Phenotypic Class
Enhanced by
Statement
Reference
NOT Enhanced by
Suppressed by
Statement
Reference
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PU has visible phenotype, suppressible by nmoadk2/nmo[+]
NOT suppressed by
Statement
Reference
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PU has visible | adult stage phenotype, non-suppressible by GIIIspla2EP1516/GIIIspla2[+]
Enhancer of
Other
Phenotype Manifest In
Enhanced by
Statement
Reference
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PU has retina phenotype, enhanceable by Diap14/th[+]
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PU has retina phenotype, enhanceable by CtBP[+]/CtBP03463
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PU has retina phenotype, enhanceable by GstS1[+]/GstS106253
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PU has eye phenotype, enhanceable by mub04093/mub[+]
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PU has eye phenotype, enhanceable by CG14438[+]/sovKG00226
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PU has retina phenotype, enhanceable by CG14438[+]/sovKG00226
NOT Enhanced by
Statement
Reference
Suppressed by
Statement
Reference
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PU has eye phenotype, suppressible by nmoadk2/nmo[+]
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PF has eye phenotype, suppressible by rl1
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PU has retina phenotype, suppressible by pumbem/pum[+]
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PU has retina phenotype, suppressible by 14-3-3εex4/14-3-3epsilon[+]
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PU has retina phenotype, suppressible by 14-3-3εj2B10/14-3-3epsilon[+]
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PU has retina phenotype, suppressible by ps[+]/ps3
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PU has retina phenotype, suppressible by ps[+]/psj449
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PU has eye phenotype, suppressible by Akt1[+]/Akt104226
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PU has retina phenotype, suppressible by Akt1[+]/Akt104226
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PF has eye phenotype, suppressible | partially by Spf45J23/Spf45[+]
NOT suppressed by
Statement
Reference
Hsap\ATXN182Q.UAS, Scer\GAL4GMR.PU has eye phenotype, non-suppressible by GIIIspla2EP1516/GIIIspla2[+]
Enhancer of
NOT Enhancer of
Suppressor of
NOT Suppressor of
Other
Additional Comments
Genetic Interactions
Statement
Reference
Coexpression of sensScer\UAS.cNa with Hsap\ATX182Q.Scer\UAS (both under the control of Scer\GAL4sca-109-68) abolishes the loss of thoracic bristles phenotype of Hsap\ATX182Q.Scer\UAS overexpression, although the number of extra bristles is decreased compared to sensScer\UAS.cNa expression alone.
Xenogenetic Interactions
Statement
Reference
Co-expression of NmnatScer\UAS.PD partially suppresses the age-dependent decline in climbing performance and signs of increased apoptosis in the brain in flies with expression of Hsap\ATXN182Q.Scer\UAS driven by Scer\GAL4nrv2.PS; co-expression of Nmnatcyt.Scer\UAS.PC has no effect, while co-expression of NmnatScer\UAS.PC enhances the decline and apoptosis.
Expression of RpA-70GS10684 suppresses the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is suppressed. Expression of RecQ5GS9658 suppresses the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is enhanced. Expression of grpGS22185 suppresses the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is enhanced. Expression of touGS16324 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of touGS18086 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of lig3GS9746 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is enhanced. Expression of CG9601GS21535 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is suppressed. Expression of Fen1GS9817 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is enhanced. Expression of mus210GS13949 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of Ercc1GS20656 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of DNA-ligIGS20555 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is enhanced. Expression of Ssl1GS20889 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of mrnGS8019 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of Mms19GS20566 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). Expression of GS16203GS16203 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is enhanced. Expression of CG6197GS51333 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is enhanced. Expression of XpdGS36 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is enhanced. Expression of CG8841GS10988 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is enhanced. Expression of rad50GS7243 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of spn-BGS13042 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is suppressed. Expression of GenGS21003 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of tefuGS20121 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of IrbpGS10368 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of mus81GS1026 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is enhanced. Expression of CG12728GS20801 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is suppressed. Expression of mms4GS22070 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is enhanced. Expression of gktGS50611 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of Pms2GS10168 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of Msh6GS13002 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of PCNAGS15324 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of mei-W68GS10670 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is enhanced. Expression of CycHGS9043 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is suppressed. Expression of DNApol-δGS20870 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of DNApol-εGS9667 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is suppressed. Expression of DNApol-ηGS15067 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is suppressed. Expression of jnjGS21013 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of perGS20805 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is suppressed. Expression of CG5181GS11373 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of UbcD6GS22499 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is not suppressed. Expression of BlmGS12928 does not suppress the reduction in eclosion rate seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4RapGAP1-OK6 (calculated using the Virtual Eclosion Rate (VER) method). The shortened lifespan is enhanced. Expression of grpGS22185 exacerbates the eye degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of grpNIG.17161R suppresses the eye degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of RpA-70GS10684 suppresses the eye degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of RpA-70NIG.9633R enhances the eye degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU.
Coexpression of TER94GS10258 partially rescues the reduced retina thickness seen in animals expressing Hsap\ATXN182Q.Scer\UAS under the control of Scer\GAL4GMR.PU results in reduced retina thickness compared to controls.
One copy of nmoadk2 strongly suppresses the retinal depigmentation and ommatidial disorganisation phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed in the eye under the control of Scer\GAL4GMR.PU. Co-expression of Hsap\NLKScer\UAS.cJa enhances the ommatidial disorganisation and retinal degeneration phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed in the eye under the control of Scer\GAL4GMR.PU. There is a dramatic increase in the number and size of the areas of necrotic cell death. Co-expression of Hsap\NLKKN.Scer\UAS.cJa has little effect on the ommatidial disorganisation and retinal degeneration phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed in the eye under the control of Scer\GAL4GMR.PU.
licKK108550 partially suppresses the external eye phenotype and photoreceptor degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF. Expression of SdrKK104654 partially suppresses the external eye phenotype and photoreceptor degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF. Expression of CG8866KK102129 partially suppresses the external eye phenotype and photoreceptor degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF. Expression of WnkKK102654 partially suppresses the external eye phenotype and photoreceptor degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF. Expression of BubR1DN.Scer\UAS partially suppresses the external eye phenotype and photoreceptor degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF. Expression of JIL-1KK101300 partially suppresses the external eye phenotype and photoreceptor degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF. The reduction in lifespan seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4nrv2.PS is also partially rescued. JIL-1KG08229 partially suppresses the external eye phenotype and photoreceptor degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF. The progressive impairment of motor performance seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4nrv2.PS is also partially rescued. Dsor1G79 partially suppresses the external eye phenotype and photoreceptor degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF. The progressive impairment of motor performance and reduction in lifespan seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4nrv2.PS are also partially rescued. rl1 partially suppresses the external eye phenotype and photoreceptor degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF. The progressive impairment of motor performance and reduction in lifespan seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4nrv2.PS are also partially rescued. Expression of CG17565KK108342 partially suppresses the external eye phenotype and photoreceptor degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF. Expression of drkKG03077 partially suppresses the external eye phenotype and photoreceptor degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF. SosKG10074 partially suppresses the external eye phenotype and photoreceptor degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF. The progressive impairment of motor performance and reduction in lifespan seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4nrv2.PS are also partially rescued. Ras85D06677 partially suppresses the external eye phenotype and photoreceptor degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF. The progressive impairment of motor performance and reduction in lifespan seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4nrv2.PS are also partially rescued. Expression of Ras64BGD1517 partially suppresses the external eye phenotype and photoreceptor degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF. Expression of phlKK100982 partially suppresses the external eye phenotype and photoreceptor degeneration seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF. The progressive impairment of motor performance and reduction in lifespan seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4nrv2.PS are also partially rescued. Expression of CG2976KK100303 partially suppresses the progressive impairment of motor performance and reduction in lifespan seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4nrv2.PS.
One copy of GIIIspla2EP1516 does not suppress the rough eye phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU.
Co-expression of two copies of P{Sym-UAS-Hsrω} restores pigmentation and reduces the roughening of Hsap\ATX182Q.Scer\UAS, Scer\GAL4GMR.PF eyes. Co-expression of a single copy of P{Sym-UAS-Hsrω} significantly lowers the proportion of Scer\GAL4elav-C155, Hsap\ATX182Q.Scer\UAS flies dying at each stage of development, such that a greater fraction die as differentiated pharates.
Expression of DnaJ-1EP411 suppresses the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of Hsc70-3K97S.Scer\UAS enhances the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. eff8 enhances the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of Hsap\STUB1Scer\UAS.cAa suppresses the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Sin3AdQ4 enhances the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. One copy of skdL7062 enhances the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of Sir2EP2300 enhances the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of taraEP3463 enhances the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of cpoEP3378 enhances the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of pumEP3461 enhances the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. One copy of pumbem suppresses the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of 14-3-3εScer\UAS.cCa enhances the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. One copy of 14-3-3εex4 suppresses the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. One copy of 14-3-3εj2B10 suppresses the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of RhoGAPp190EP1357 enhances the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of thScer\UAS.T:Hsap\MYC.cHa suppresses the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. One copy of th4 enhances the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. One copy of Hsrωneo55 enhances the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. One copy of Sc205634 enhances the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. One copy of Rpd304556 enhances the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. One copy of CtBP03463 enhances the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. One copy of ps3 suppresses the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. One copy of psj449 suppresses the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. One copy of GstS106253 enhances the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of Nup44AEP2417 suppresses the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of Akt1Scer\UAS.T:Ivir\HA1 enhances the external eye and retinal phenotypes seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. One copy of Akt104226 suppresses the external eye and retinal phenotypes seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of mubEP3623 suppresses the external eye and retinal phenotypes seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. One copy of mub04093 enhances the external eye and retinal phenotypes seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of vibEP651 suppresses the external eye and retinal phenotypes seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of vibEP513 suppresses the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of CG14438EP1438 suppresses the external eye and retinal phenotypes seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. One copy of CG14438KG00226 enhances the external eye and retinal phenotypes seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of Pi3K92EScer\UAS.T:Hsap\MYC enhances the retinal phenotype seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PU. Expression of mubEP3623 partially suppresses the climbing defects seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4nrv2.PS. Expression of vibEP651 partially suppresses the climbing defects seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4nrv2.PS. Expression of CG14438EP1438 increases the number of neurons that contain nuclear inclusions seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4ey-OK107. One copy of CG14438KG00226 reduces the number of neurons that contain nuclear inclusions seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4ey-OK107. Expression of vibEP651 reduces the number of neurons that contain nuclear inclusions seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4ey-OK107. Expression of vibEP513 reduces the number of neurons that contain nuclear inclusions seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4ey-OK107. Expression of Hsap\STUB1Scer\UAS.cAa reduces the number of neurons that contain nuclear inclusions seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4ey-OK107.
One copy of Spf45J23 partially suppresses the ommatidial disorganisation and interommatidial bristle loss seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF at 30[o]C. Expression of Hsap\RBM17Scer\UAS.cLa enhances the ommatidial disorganisation and bristle loss seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF at 25[o]C. Expression of Spf45Scer\UAS.cLa enhances the ommatidial disorganisation and bristle loss seen when Hsap\ATXN182Q.Scer\UAS is expressed under the control of Scer\GAL4GMR.PF at 25[o]C.
The rough eye and rhabdomere phenotypes of Scer\GAL4GMR.PF>Hsap\ATXN182Q.Scer\UAS are partially suppressed by co-expression of NmnatScer\UAS.cZa. The rough eye and rhabdomere phenotypes of Scer\GAL4GMR.PF>Hsap\ATXN182Q.Scer\UAS are partially suppressed by co-expression of NmnatWR.Scer\UAS.
The defective adult eye morphology (disorganization of ommatidia and loss of bristles, retinal tissue loss, photoreceptor cell defects) observed in flies expressing either Hsap\ATXN182Q.Scer\UAS or Atx2Scer\UAS.cSa under the control of Scer\GAL4GMR.PU are exacerbated further when the two transgenes are co-expressed. The eye defects in Hsap\ATXN182Q.Scer\UAS expressing flies are partially suppressed by combination with a single copy of Atx2X1. This suppression of the eye phenotype can be reverted (and the phenotype worsened again) by combination with Atx2Scer\UAS.cSa. The age-progressive locomotor impairment and shorter adult life span characteristic for flies expressing Hsap\ATXN182Q.Scer\UAS under the control of Scer\GAL4nrv2.PU as well as the loss of macrochaeta observed in flies expressing Hsap\ATXN182Q.Scer\UAS under the Scer\GAL4sca-109-68 driver are significantly improved by combination with a Atx2X1 in a heterozygous state. The mild loss of macrochate observed in flies expressing Hsap\ATXN182Q.Scer\UAS under the control of Scer\GAL4sca-109-68 is strongly exacerbated by co-expression of Atx2Scer\UAS.cSa.
Co-expression of Hsap\STUB1Scer\UAS.cAa suppresses the eye phenotype caused by expression of Hsap\ATX182Q.Scer\UAS under the control of Scer\GAL4GMR.PF, with the degree of suppression depending on the level of Hsap\STUB1Scer\UAS.cAa expression.
Coexpression of cicScer\UAS.cLa and Hsap\ATX182Q.Scer\UAS, both under the control of Scer\GAL4GMR.PF, results in a milder eye phenotype than expression of Hsap\ATX182Q.Scer\UAS alone; there is less ommatidial disorganization and fusion and the interommatidial bristles are partially restored. A cicD49/+ background causes worsened ommatidial disorganization of Scer\GAL4GMR.PF>Hsap\ATX182Q.Scer\UAS-expressing eyes, when flies are raised at room temperature. Scer\GAL4C5-driven expression of Hsap\ATX182Q.Scer\UAS in a cicD49/+ background results in more prominent L3 vein thickening and increased ectopic wing vein formation in the posterior part of the interveins compared to expression of Hsap\ATX182Q.Scer\UAS in a wild-type background. Coexpression of cicScer\UAS.cLa and Hsap\ATX182Q.Scer\UAS, under the control of Scer\GAL4C5, results in restoration of the posterior crossvein, which is missing in flies expressing either transgene separately.
The addition of 14-3-3εScer\UAS.cCa enhances the eye phenotypes seen in Hsap\ATX182Q.Scer\UAS, Scer\GAL4GMR.PF animals alone. They have profoundly disordered ommatidia, a thin and disorganised retina layer, and grossly abnormal rhabdomeres. Animals expressing Akt1Scer\UAS.T:Ivir\HA1 and Hsap\ATX182Q.Scer\UAS (under the control of Scer\GAL4GMR.PF) have a much stronger eye phenotype than seen in either Akt1Scer\UAS.T:Ivir\HA1 or Hsap\ATX182Q.Scer\UAS on their own. Profoundly disorganised ommatidia and numerous necrotic spots are seen, as well a severely disorganised retina with missing or malformed rhabdomeres. The addition of Dp(3;Y)L58 to animals expressing Akt1Scer\UAS.T:Ivir\HA1 (under the control of Scer\GAL4GMR.PF) enhances the eye neurodegeneration phenotype. The addition of Pi3K92EScer\UAS.T:Hsap\MYC (driven by Scer\GAL4GMR.PF) dramatically worsens the Hsap\ATX182Q.Scer\UAS neurodegeneration phenotype in the eye. the addition of Pk61CEP3553 (driven by Scer\GAL4GMR.PF) has no effect on the neurodegeneration phenotype in the eye. The addition of Akt104226 to animals expressing Akt1Scer\UAS.T:Ivir\HA1 (under the control of Scer\GAL4GMR.PF) suppresses the eye neurodegeneration phenotype.
Expression of Hsap\ATX130Q.Scer\UAS when driven by Scer\GAL4GMR.PF can lead to a severe degeneration of the retina. When driven by Scer\GAL4VNC.ap, a progressive elimination of the cell bodies and axonal projections of interneurons. The addition of DnaJ-1EP411 to Hsap\ATX182Q.Scer\UAS flies produces a suppression of the eye phenotype, revealing less disrupted ommatidia and improved retinal thickness.
Complementation and Rescue Data
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Hsap\ATX182Q.Scer\UAS
Hsap\ATXN182Q.Scer\UAS
Hsap\ATXN182Q.UAS
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