A Database of Drosophila Genes & Genomes

FB2013_03, released May 7th, 2013
 

Allele Hsap\ATXN3tr.Q78.Scer\UAS.T:Ivir\HA1

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SymbolHsap\ATXN3tr.Q78.Scer\UAS.T:Ivir\HA1SpeciesH. sapiens
NameFlyBase IDFBal0090630
Feature typealleleAssociated geneHsap\ATXN3
Allele class
Mutagenin vitro construct - regulatory fusionin vitro construct - coding region fusion
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Construct: A truncated form of Hsap\MJD, consisting of an expanded length glutamine tract (78 repeats) flanked upstream by 12 amino acids and downstream by the carboxy terminal 43 amino acids of Hsap\MJD, is expressed under the control of Scer\UAS regulatory sequences. The Hsap\MJD protein is tagged at the N-terminal end with T:Ivir\HA1.
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Flies expressing Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.long] have a rough eye phenotype.
Expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[ninaE.PT] results in inclusion formation, a decrease in polyQ protein solubility and progressive neural loss. At 21 days the number of photoreceptors per ommatidium is reduced in male flies compared to controls.
Expression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] in the developing eye under the control of Scer\GAL4[GMR.PF] induces clear defects in the eye, with defective ommatidia seen in 18% of cases.
When Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] expression is induced 'early', only for 4 days after eclosion (using the 'TARGET' system and Scer\GAL4[GMR.PF] and Scer\GAL80[ts.αTub84B]), rhabdomere loss is not observable until 40 days of age. However, when Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] expression os induced from 20 to 24 days of age (i.e. 'late'), neurodegeneration with rhabdomere loss of the ommatidia is observable at 40 days of age, suggesting that Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] expression in older flies causes more severe neurodegeneration than expression in younger flies.
Overexpression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU] results in massive degeneration of retinal cells giving rise to rough and de-pigmented eyes in freshly eclosed flies. In 50 h old (mid pupal stage) eye discs expressing Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] the integrity of photoreceptor cells is poor when compared with wild-type pupal retina. Small vacuoles are present in Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] expressing retinas and the characteristic arrangement of the seven photoreceptors and the actin filaments within them are also altered. Unlike wild-type controls, flies overexpressing Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU] do not show any significant phototaxis. Pan-neuronal overexpression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] via Scer\GAL4[elav.PU] results in 100% lethality at pupal stage.
Expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PF] results in degeneration and depigmentation of the eye.
Flies expressing Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] using Scer\GAL4[GMR.PF] show progressive eye degeneration, visualized by the presence of dark patches and collapsed eyes.
Pan-neural expression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] (under the control of Scer\GAL4[elav-C155]) causes age-dependent neurodegeneration, as quantified by decreased numbers of Kenyon cells in the mushroom body.
Expression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] in the eye under the control of Scer\GAL4[GMR.PU] results in eye degeneration, and patched, speckled, and collapsed eye phenotypes are observed.
Flies expressing Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PF] display a rough-eye phenotype.
Expression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] using Scer\GAL4[GMR.PF] induces substantial degeneration in eyes with loss of pigmentation and eye morphology. 2-day old flies expressing Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] using Scer\GAL4[GMR.PF] fail to detect light.
One day old flies expressing a strong insertion of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PF] show severe compound eye degeneration. This degeneration is strongly suppressed upon treatment with the HSF1-activating compound 17-AAG at concentrations of 500nM, 1.5µM and 5µM. Treatment at 50nM shows only a moderate therapeutic effect and treatment at 50µM has no effect. The suppression seen with 17-AAG is stronger than is seen with the histone deacetylase inhibitor SB, which shows only a weak effect. Treatment with GA at concentrations of 500nM and 5µM also results in modest suppression of compound eye degeneration, but no suppression is seen at concentrations of 500nM and 50µM. Treatment with RA at concentrations ranging from 50nM to 5µM also effectively suppresses neurodegeneration. No detectable changes are seen when the flies are treated with either CL (1 to 100 µM) or GGA (10µM to 40 mM). Expression of a strong insertion of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] in the nervous system under the control of Scer\GAL4[elav.PLu] significantly decreases the survival rate of flies during their development to adults. This lethality is significantly suppressed upon treatment with the HSF1-activating compound 17-AAG. Eye discs from flies expressing a weak insertion of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PF] contain numerous inclusion bodies in the region posterior to the morphogenetic furrow. Significantly fewer inclusion bodies are seen when the flies are treated with the HSF1-activating compound 17-AAG and the size of the remaining inclusion bodies is significantly reduced. Treatment with 17-AAG also reduces the ratio of the anteroposterior width of the area with cells containing inclusion bodies to that of the area with cells containing diffusely distributed protein.
Flies expressing Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] in the developing eye under the control of Scer\GAL4[GMR.PF] have a normal external eye, although internally the retina is disorganised. Flies expressing Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] in differentiated photoreceptor neurons under the control of Scer\GAL4[ninaE.PD] are born with the normal complement of seven visible photoreceptors per ommatidium, but progressive degeneration occurs, reducing the number of photoreceptors to on average 6.4 per ommatidium by 12 days and 3.7 by 18 days. Fewer neuron clones expressing Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] (generated under the control of Scer\GAL4[elav-C155] using MARCM) are retained compared to wild type. Only 13% are still detectable at 3 days and none are recovered at 6 days. This compares to an 83% recovery rate in controls. In retinal sections of animals expressing Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] in differentiated photoreceptor neurons under the control of Scer\GAL4[ninaE.PD] inclusions are sparse at 24h but prominent by 4 days.
One day old flies expressing Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[elav-C155] show mild loss of retinal integrity, with approximately 5.8 photoreceptors per ommatidium, rather than the usual seven seen in controls. The lifespan of these flies is reduced compared to controls. Photoreceptor loss is also seen in 25 day old flies expressing Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] in differentiated photoreceptors under the control of Scer\GAL4[ninaE.PD]; these flies have on average 4.6 photoreceptors per ommatidium. Flies expressing Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] pan-neuronally under the control of Scer\GAL4[elav-C155] show normal climbing behaviour at one day old. Ability to climb becomes progressively worse with age, with approximately 42.1 percent of flies unable to climb by 12 days.
Expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU] results in external and internal degeneration of the eye, with pigment loss and severely reduced retinal thickness. 1 day old flies expressing Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU] are functionally blind in a phototaxis assay, distributing randomly between a light and dark chamber.
Expression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] driven by Scer\GAL4[GMR.PF] results in ablation of the adult eye.
50 day old flies expressing Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4elav.PLu show almost no climbing activity.
Expression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] in the eye (under the control of Scer\GAL4[GMR.PF]) induces degeneration, characterised by partial loss of pigmentation and retinal structure.
Expression of Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1, under the control of Scer\GAL4GMR.PF, induces progressive degeneration of photoreceptors, coupled with the formation of protein aggregates in 2-day old flies.
Expression of a 'strong' expression line of Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4tim.PE disables circadian locomotor activity patterns. However, overall locomotor levels are not significantly different from wild type. Expression of a 'strong' expression line of Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4Ddc.PL causes no detectable circadian abnormality but does cause defects in female mating latency. Expression of a 'strong' expression line of Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4tim.PE results in a significant decrease in the number of Pdf-positive small ventral latero-neurons, whereas the number of Pdf-positive large ventral latero-neurons is unaffected. Flies expressing Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4tim.PE have no gross morphological abnormalities. Both male and female flies expressing Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4tim.PE show a significant decrease in mating activity (during a 1 hour observation period); fertility of these flies is unaffected.
Expression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] in the brain under the control of Scer\GAL4[30Y] induces apoptotic neurodegeneration in the absence of cell cycle activation. Cortical neuron loss and neuropil rarefaction is observed in these animals. Expression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] in the eye under the control of Scer\GAL4[GMR.PF] results in a rough-eye phenotype.
Expression of Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF results in roughened, depigmented eyes which contain black necrotic spots and are collapsed. Expression of Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4elav.Switch.PO in the presence of RU486 results in a reduction in adult life span compared to control flies (which have not been fed RU486).
Targeted expression of Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 in neurons using the Scer\GAL4elav-C155 driver results in early adult death. Ectopic expression of Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1, driven by Scer\GAL4GMR.PF, leads to late-onset degeneration in adult eyes. Eyes show loss of retinal structure and lack of pigment one day after eclosion.
Expression of Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF results in severe degeneration of the retina that is associated with severe collapse of the eye. Co-expression of Hsap\MJDfl.Q27.Scer\UAS.T:Hsap\MYC restores the eye and retina phenotypes towards normal in flies expressing Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF. Co-expression of Hsap\MJDtr.Q27.Scer\UAS.T:Ivir\HA1 does not suppress the eye degeneration phenotype caused by Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF. Co-expression of Hsap\MJDΔ.Scer\UAS.T:Hsap\MYC suppresses the eye degeneration phenotype caused by Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF. Co-expression of Hsap\MJDfl.Q27.UIMmut.Scer\UAS.T:Hsap\MYC partially suppresses the eye degeneration phenotype caused by Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF. Co-expression of Hsap\MJDfl.Q27.C14A.Scer\UAS.T:Hsap\MYC does not suppress the eye degeneration phenotype caused by Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF. Co-expression of Hsap\MJDfl.Q84.Scer\UAS.T:Hsap\MYC partially suppresses the eye degeneration phenotype caused by Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF.
Expression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PF] results in a retinal degeneration phenotype. Expression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[30Y] results in a brain degeneration phenotype; neurons are present in normal numbers in 1 day old flies, but approximately 50% of Kenyon cells degenerate by 20 days after eclosion.
When Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 driven by Scer\GAL4elav-C155 nuclear aggregates are seen.
Animals expressing Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 driven by Scer\GAL4179Y or Scer\GAL4Appl.G1a exhibit a severe sluggish larval movement phenotype. A large increase in neuronal apoptosis is seen. When Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 is overexpressed at 29oC larvae do not survive to adulthood but die at second or third instar larval stage.
When Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 us driven by Scer\GAL4GMR.PF leads to severe, progressive eye degeneration contributing to abnormal ommatidium morphology.
Flies expressing Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF have a severe eye degeneration phenotype, with loss of external and internal eye structure. The degeneration becomes more severe over the lifetime of the adult fly.
Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1; Scer\GAL4GMR.PF flies have an eye neurodegeneration phenotype.
Expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU] results in severe degeneration of the eye, with loss of external pigmentation and severe loss of photoreceptor cells in the retina. Weaker expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU] results in a progressive degeneration of the retina as the adults age.
Strong expression of Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF results in a lack of eye pigmentation, indicating loss of pigment cells, and a collapsed eye, indicating loss of photoreceptor neurons. Weaker expression of Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF causes progressive degeneration of the eye during adult life.
Flies expressing Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF have degenerate eyes that lack pigment and show severe loss of retinal structure. Expression of Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4elav-C155 results in complete lethality or early adult death. Progressive degeneration of the eye, seen as disruption of the regular rhabdomere pattern, is seen.
Flies expressing Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF have rough eyes. The severity of the phenotype varies from mild roughness of the eye, through loss of pigment with severely disrupted eye morphology, to reduced eye size. The retina shows cell degeneration. The lamina and medulla show good structure and are normal in size, and the optic chiasm is present, even in flies with a severe phenotype. Large holes are visible in the most lateral part of the lamina, due to separation of the retinal basement membrane from the lamina in flies with moderate or severe phenotypes. The eye morphology progressively degenerates as the flies age. The retina does not deepen as much as normal in pupae expressing Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF. Pigmentation initiates at the correct time, but only occurs round the periphery of the developing eye. Flies expressing Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4elav-C155 emerge but show an early death phenotype in weak or moderate expressing P{UAS-Hsap\MJD.tr-Q78} lines. The brains of 4 day old flies appear shrunken compared to 1 day old flies, with the flies dying by 5 days. Gross degeneration of the brain is not seen. The eyes show some irregularity and loss of cell morphology of the rhabdomere lattice in 1 day old flies. A much greater loss of cell integrity is seen in 4 day old flies. Flies expressing Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4elav-C155 in strong expressing P{UAS-Hsap\MJD.tr-Q78} lines do not survive to adulthood. Flies expressing Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4how-24B die as embryos or larvae. Expression of Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4dpp.blk1 has no phenotypic effect.
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Hsap\ATXN3tr.Q78.Scer\UAS.T:Ivir\HA1, Scer\GAL4ninaE.PT has adult brain | conditional phenotype, non-suppressible by Eip74EF[+]/Eip74EFBG01805
Hsap\ATXN3tr.Q78.Scer\UAS.T:Ivir\HA1, Scer\GAL4ninaE.PT has ommatidium | conditional phenotype, non-suppressible by Eip74EF[+]/Eip74EFBG01805
Hsap\ATXN3tr.Q78.Scer\UAS.T:Ivir\HA1, Scer\GAL4ninaE.PT has photoreceptor | conditional phenotype, non-suppressible by Eip74EF[+]/Eip74EFBG01805
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The rough eye phenotype caused by expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.long] is suppressed by co-expression of Hsap\HSPA1L[Scer\UAS.cWa], but is not suppressed by co-expression of Hsap\TRAP1[Scer\UAS.cBa].
Expression of mir-34[Scer\UAS.cLa] partially suppresses the degeneration seen when Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] is expressed under the control of Scer\GAL4[ninaE.PT]. Inclusion formation is slowed, the protein retains greater solubility and neural degeneration is suppressed. The reduced number of photoreceptors per ommatidium seen in 21 day old flies is also partially rescued. One copy of Eip74EF[BG01805] has little effect on the degeneration seen when Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] is expressed under the control of Scer\GAL4[ninaE.PT].
Co-expression of Plip[GD4965] under the control of Scer\GAL4[ninaE.PT] has no significant effect on Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1]-induced photoreceptor cell degradation. Co-expression of Plip[KK108325] under the control of Scer\GAL4[ninaE.PT] has no significant effect on Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1]-induced photoreceptor cell degradation.
Expression of Mmus\Prnp[P101L.Scer\UAS.T:Hsap\PRNP-3F4] significantly enhances the pathogenic eye defects seen upon expression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] in the eye (both lines under the control of Scer\GAL4[GMR.PF]), with approximately 53% of ommatidia displaying aberrant structures.
Co-expression of Hsp60D[dsRNA.Sym.Scer\UAS] with Scer\GAL4[GMR.PU]-driven Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] suppresses the eye degeneration phenotype resulting from Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] overexpression. The eyes show normal pigmentation and more or less regularly organised ommatidial arrays nearly comparable to those in wild-type eyes. Co-expression of Hsp60D[dsRNA.Sym.Scer\UAS] with Scer\GAL4[GMR.PU]-driven Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] restores phototaxis. Co-expression of Hsp60D[dsRNA.Sym.Scer\UAS] via Scer\GAL4[elav.PU] partially suppresses the lethality resulting from Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] overexpression. The escapers are short lived with a maximum life span of six days. Co-expression of Pros26[1.Scer\UAS] via Scer\GAL4[elav.PU] does not significantly affect the Hsp60D[dsRNA.Sym.Scer\UAS]-mediated partial suppression of the eye degeneration phenotype caused by Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] overexpression. The Hsp60D[dsRNA.Sym.Scer\UAS]-mediated suppression of the eye degeneration phenotype caused by Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] overexpression via Scer\GAL4[GMR.PU] is not much affected by heterozygosity for th[5]. Co-expression of Hsp60D[dsRNA.Sym.Scer\UAS] via Scer\GAL4[GMR.PU] fails to suppress the eye degeneration in Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] and th[dsRNA.Scer\UAS] expressing flies. The eye phenotype resulting from simultaneous expression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] and Hsp60D[dsRNA.Sym.Scer\UAS] via Scer\GAL4[GMR.PU] does not appear to be affected by co-expression of Uba2[C175S.Scer\UAS]. The eye degeneration caused by Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] overexpression via Scer\GAL4[GMR.PU] is enhanced by co-expression of Pros26[1.Scer\UAS]. Heterozygosity for th[5] enhances the eye degeneration resulting from the overexpression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] via Scer\GAL4[GMR.PU]. Co-expression of th[dsRNA.Scer\UAS] via Scer\GAL4[GMR.PU] in Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1]-expressing flies results in enhanced eye degeneration with reduction in eye size. Simultaneous expression of Uba2[C175S.Scer\UAS] and Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] via Scer\GAL4[GMR.PU] enhances eye degradation, resulting in more glazed eyes with little pigmentation.
Scer\GAL80[Scer\FRT.αTub84B] has no effect on the eye degeneration and depigmentation caused by expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PF]. When FLPase is used to remove the Scer\FRT cassette in Scer\GAL80[Scer\FRT.αTub84B], generating Scer\GAL80[αTub84B.PB] which expresses Scer\GAL80, the eye phenotype caused by expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PF] is completely suppressed. Flies carrying Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1], Scer\GAL4[GMR.PF] and Scer\GAL80[αTub84B.PG] have normal eyes, due to repression of Scer\GAL4[GMR.PF] expression by Scer\GAL80[αTub84B.PG]. This repression can be removed using FLPase (which removes the Scer\FRT cassette containing the Scer\GAL80 coding region from Scer\GAL80[αTub84B.PG]).
Co-expression of Hsap\HSPB8[Scer\UAS.WT] or Hsp67Bc[Scer\UAS.T:SV5\V5] significantly decreases the eye degeneration seen in Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1], Scer\GAL4[GMR.PF] flies. Co-expression of Hsp67Bc[GD11244] significantly worsens the eye degeneration seen in Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1], Scer\GAL4[GMR.PF] flies. Co-expression of Hsap\HSPB8[Scer\UAS.K141E] has no significant effect on the eye degeneration phenotype seen in Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1], Scer\GAL4[GMR.PF] flies. Co-expression of Hsap\HSPB8[Scer\UAS.K141N] significantly decreases the eye degeneration seen in Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1], Scer\GAL4[GMR.PF] flies.
Removing cathD from mutant Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] overexpressing flies (under the control of Scer\GAL4[elav-C155] in a cathD[1] background) has no effect on Kenyon cell loss.
Expression of nej[F2161A.Scer\UAS.T:SV5\V5] in the Scer\GAL4[GMR.PF], Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] background results in 15% lethality at the pupal stage. Surviving flies show a reduction in eye size and a greater disruption in ommatidial arrays. Additional co-expression of one copy of P{Sym-UAS-Hsrω} improves the eye morphology and size and reduces pupal death. Expression of nej[dsRNA.Scer\UAS.cKa] in the Scer\GAL4[GMR.PF], Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] background results in >97% lethality at the pharate stage with eye degeneration being dramatically enhanced. Additional co-expression of P{Sym-UAS-Hsrω} robustly suppresses this enhanced eye damage, and also substantially reverses Scer\GAL4[GMR.PF], Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] eye degeneration in a dose-dependent manner. Co-expression of P{Sym-UAS-Hsrω} also improves emergence to adult stage in a dose-dependent manner. The damaged eye phenotype that results from Scer\GAL4[GMR.PF]-mediated expression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] is suppressed by co-expression of P{Sym-UAS-Hsrω}, and is not further enhanced by co-expression of Pros26[1.B.Scer\UAS] and Prosβ2[1.Scer\UAS]. Co-expression of P{Sym-UAS-Hsrω} still suppresses the damage in Scer\GAL4[GMR.PF], Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1], Pros26[1.B.Scer\UAS], Prosβ2[1.Scer\UAS] eyes, but the eyes are not rescued to the degree seen in Scer\GAL4[GMR.PF], Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] eyes.
Expression of Hsap\HSPB7[Scer\UAS.cVa] in flies expressing Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU] suppresses the eye degeneration phenotype.
Expression of Hsap\MJD[Q27.Scer\UAS.T:Hsap\MYC.T:Zzzz\FLAG] in flies expressing Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PF] suppresses the eye phenotype. Expression of Hsap\MJD[Q84.Scer\UAS.T:Hsap\MYC.T:Zzzz\FLAG] in flies expressing Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PF] partially suppresses the eye phenotype. Expression of Hsap\MJD[6M.Q83.Scer\UAS.T:Hsap\MYC.T:Zzzz\FLAG] in flies expressing Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PF] partially suppresses the eye phenotype.
Co-expression of two copies of P{Sym-UAS-Hsrω} almost completely suppresses the Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1], Scer\GAL4[GMR.PF] eye phenotype: the external appearance is wild type but rhabdomere arrays are still abnormal. Co-expression of one or two copies of P{Sym-UAS-Hsrω} restores normal phototactic behaviour to Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1], Scer\GAL4[GMR.PF] flies. Co-expression of a single copy of P{Sym-UAS-Hsrω} rescues the pharate lethality seen in Scer\GAL4[elav-C155], Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] flies, allowing 94% of pupae to eclose. However, surviving female flies display abdominal swellings, and lifespan is reduced compared to controls.
Expression of Hsf[dsRNA.Scer\UAS] suppresses the ability of the HSF1-activating compound 17-AAG to rescue the degeneration seen in flies expressing a strong insertion of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PF]. The amount of degeneration seen is similar to that observed in untreated flies. However, expression of Hsf[dsRNA.Scer\UAS] also slightly enhances the compound eye degeneration in the absence of 17-AAG.
Co-expression of Atx2[Scer\UAS.cSa] and Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] in the developing eye under the control of Scer\GAL4[GMR.PF] produces a more severe phenotype than in either mutant alone. The flies exhibit strong eye degeneration with loss of pigmentation and severe collapse of the retina. Co-expression of Atx2[Scer\UAS.cSa] and Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] in differentiated photoreceptor neurons under the control of Scer\GAL4[ninaE.PD] enhances the photoreceptor loss seen when either construct is expressed alone. Flies are still born with the normal complement of seven visible photoreceptors, but whereas at six days the single mutants appear near normal, co-expression results in only 4.7 photoreceptors per ommatidium. At 18 days almost complete photoreceptor loss (1.3 photoreceptors per ommatidium) is seen. One copy of Atx2[X1] suppresses the eye degeneration seen when Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] is expressed in differentiated photoreceptor neurons under the control of Scer\GAL4[ninaE.PD]. On average 6.9 visible photoreceptors are seen per ommatidium in 12 day old flies, similar to the 7 seen in controls. This compares to 6.4 when Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] is expressed alone. Homozygous Atx2[X1] suppresses the neuron loss seen in anterior wing margin clones induced in flies expressing Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[elav-C155] (generated using MARCM). 77% of neurons are retained at 3 days, compared to only 13% when Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] is expressed alone. 40% are recovered at 6 days, in contrast to the complete loss of neurons in Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] expressing flies. Expression of Atx2[Scer\UAS.cSa] enhances the rate of inclusion formation seen when Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] is expressed in differentiated photoreceptor neurons under the control of Scer\GAL4[ninaE.PD]. Inclusions are more prominent at 24 hours, but by four days appear similar to when Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] is expressed alone. Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] has no effect on the eye phenotype seen when Atx2[ΔC1.Scer\UAS] is expressed in the developing eye under the control of Scer\GAL4[GMR.PF]. The phenotype seen is identical to that seen when Atx2[ΔC1.Scer\UAS] is expressed alone. Co-expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] and Atx2[ΔC1.Scer\UAS] in differentiated photoreceptor neurons under the control of Scer\GAL4[ninaE.PD] causes mild photoreceptor loss at six days (an average of 6.7 visible photoreceptors per ommatidium, compared to seven in controls). This is similar to the phenotype seen when Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] is expressed alone. Expression of Atx2[ΔC1.Scer\UAS] does not enhance the rate of inclusion formation seen when Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] is expressed in differentiated photoreceptor neurons under the control of Scer\GAL4[ninaE.PD]. One copy of Df(2R)ED3610 enhances the photoreceptor loss seen when Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] is expressed in differentiated photoreceptor neurons under the control of Scer\GAL4[ninaE.PD]. On average 1.6 photoreceptors are seen per ommatidium in 18 day old flies, as opposed to the seven seen in wild type. This compares to 3.5 when Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] is expressed alone. Expression of pAbp[Scer\UAS.cSa] partially suppresses the photoreceptor loss seen when Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] is expressed in differentiated photoreceptor neurons under the control of Scer\GAL4[ninaE.PD]. On average 6.0 visible photoreceptors are seen per ommatidium in 18 day old flies, as opposed to the seven seen in wild type. This compares to 4.2 when Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] is expressed alone.
Expression of mbl[Scer\UAS.cLa] enhances the reduction in lifespan and photoreceptor loss seen when Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] is expressed pan-neuronally under the control of Scer\GAL4[elav-C155]. Flies have on average 3.0 photoreceptors per ommatidium, compared to the usual seven. One copy of mbl[E27] partially suppresses the shortened lifespan seen when Hsap\ATXN3[tr.Q61.Scer\UAS.T:Ivir\HA1] is expressed in the developing eye under the control of Scer\GAL4[GMR.PF].
Hsp68[E407], mrj[E1050] and CG14207[EP1348] are each strong suppressors of the eye degeneration phenotype caused by expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU]. External eye pigmentation is restored, and the thickness of the retina is restored towards normal. DnaJ-1[B345.2], DnaJ-1[EP411], Tpr2[EB7-1A], Ubp64E[E213-1A], wech[JM120], wech[JM265], wech[E546], wech[EP2594], Imp[EP1433] and CG5009[B227.2] are each strong suppressors of the eye degeneration phenotype caused by expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU]. CG11700[EP1384], CG8209[B3-Sa], Faf[E659], emb[E2-1A], emb[E128-1A], NFAT[EP1335] and NFAT[EP1508] are each moderate suppressors of the eye degeneration phenotype caused by expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU]. Sin3A[B9-E], dbr[EP456], dbr[EP9] and orb2[B8-S] are each weak suppressors of the eye degeneration phenotype caused by expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU]. Kdm2[EP3093] is an enhancer of the eye degeneration phenotype caused by expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU]. Expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU] in the presence of Df(3L)CH20/+ results in lethality and causes severe eye degeneration in pupae. Df(2L)r10 and Df(2L)PM44 each dominantly enhance the eye degeneration phenotype caused by expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU]. Co-expression of mrj[E1050] completely restores normal visual behaviour, and co-expression of CG14207[EP1348] partially restores visual behaviour in 1 day old flies expressing Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU] and tested in a phototaxis assay. Co-expression of Prosβ6[1.B.Scer\UAS] does not enhance the eye degeneration phenotype caused by expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU]. In the presence of Prosβ6[1.B.Scer\UAS], CG11700[EP1384] cannot suppress the eye degeneration phenotype caused by expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU]. In the presence of Prosβ6[1.B.Scer\UAS], Ubp64E[E213-1A] cannot suppress the eye degeneration phenotype caused by expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU]. DnaJ-1[B345.2] and wech[JM265] are each still able to suppress the eye degeneration phenotype caused by expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU] when the flies also carry Prosβ6[1.B.Scer\UAS].
The severe reduction in climbing ability seen in 50 day old flies expressing Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4elav.PLu is partially suppressed by co-expression of one of TrxTScer\UAS.cUa, TrxTD26A.K57I.Scer\UAS, TrxTC35A.Scer\UAS, Trx-2Scer\UAS.cUa or dhdScer\UAS.cUa.
Eye degeneration due to expression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] in the eye (under the control of Scer\GAL4[GMR.PF]) is enhanced in a loqs[f00791] background, resulting in a severely degenerated eye with complete loss of pigmentation. Expression of ban[D.Scer\UAS.T:Avic\GFP-EGFP], bearing only a 100bp region containing the ban miRNA suppresses degeneration in Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] Scer\GAL4[GMR.PF] flies, restoring external and internal eye structures towards normal. Expression of ban[B.Scer\UAS.T:Avic\GFP-EGFP] does not suppress eye degeneration in Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] Scer\GAL4[GMR.PF] flies. Eye degeneration due to expression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] in the eye (under the control of Scer\GAL4[GMR.PF]) is enhanced in a Dcr-1[Q1147X] background, resulting in a severely degenerated eye with complete loss of pigmentation. Eye degeneration due to expression of Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] in the eye (under the control of Scer\GAL4[GMR.PF]) is unaffected by a Dcr-2[L811fsX] background. Co-expression of ban[B90.1] with Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] (under the control of Scer\GAL4[GMR.PF] gives normal pigementation and improved retinal structure.
Co-expression of Hsap\VCPScer\UAS.cBa with Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1, both under the control of Scer\GAL4GMR.PF fails to suppress the Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 photoreceptor phenotype in 2-day old flies.
The rough-eye phenotype observed in animals expressing Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] in the eye under the control of Scer\GAL4[GMR.PF] is not modified by co-expression of Rheb[AV4]. The rough-eye phenotype observed in animals expressing Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] in the eye under the control of Scer\GAL4[GMR.PF] is not modified by co-expression of gig[ΔAkt-P.Scer\UAS.T:Zzzz\FLAG]. The rough-eye phenotype observed in animals expressing Hsap\MJD[tr.Q78.Scer\UAS.T:Ivir\HA1] in the eye under the control of Scer\GAL4[GMR.PF] is not modified by co-expression of dap[Scer\UAS.cdNa] and Rbf[Scer\UAS.cDa].
Co-expression of either MlfScer\UAS.cFa or MlfScer\UAS.cKa results in a strong suppression of the mutant eye phenotype caused by expression of Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF. The reduction in life span that is seen in flies expressing Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4elav.Switch.PO in the presence of RU486 is partially rescued by co-expression of either MlfScer\UAS.cFa or MlfScer\UAS.cKa. The reduction in life span that is seen in flies expressing Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4elav.Switch.PO in the presence of RU486 is not modified by the addition of MlfΔC1/+ or MlfΔ5-3/+.
The eye phenotype caused by expressing Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF, is not suppressed by coexpression of Hsap\BoatScer\UAS.T:Zzzz\FLAG.
Flies expressing both Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 and debclScer\UAS.T:Ivir\HA1 in their neurons, under the control of Scer\GAL4elav-C155, show a significantly shorter lifespan than flies expressing Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 alone. Coexpression of Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 and debclScer\UAS.T:Ivir\HA1, driven by Scer\GAL4GMR.PF, accelerates the onset of eye neurodegeneration seen in adults expressing Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1. The loss of retinal structure and pigment defects are strongly enhanced by debclScer\UAS.T:Ivir\HA1 expression. Overexpression of BuffyScer\UAS.cQa enhances the Scer\GAL4GMR.PF;Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1-induced eye neurodegeneration and lethality. Eyes coexpressing debclScer\UAS.T:Ivir\HA1, BuffyScer\UAS.cQa, and Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 (all driven by Scer\GAL4GMR.PF) show a significantly weaker degenerative eye phenotype than eyes expressing BuffyScer\UAS.cQa and Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1. Ectopic expression of debclGS2263, under the control of Scer\GAL4GMR.PF, suppresses the Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1-induced neurodegeneration of the eye. In addition , debclGS2263 markedly rescues the early adult death of flies expressing Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 in their neurons, driven by Scer\GAL4elav-C155.
Co-expression of Pros261.Scer\UAS suppresses the ability of Hsap\MJDfl.Q27.Scer\UAS.T:Hsap\MYC to restore the eye and retina phenotypes towards normal in flies expressing Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF, such that the triple mutant flies have a reduction in eye pigmentation, show collapse of the eye and loss of internal retinal tissue.
When Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 is driven in neurons either by Scer\GAL4Appl.G1a or Scer\GAL4179Y, and combined with Khc9, mutant larval nerves contain axonal blockages. Mutant nerves also contain enlarged axons, some almost four or five times the diameter of those seen in wild-type. Sometimes "holes" lacking organelles are seen within the nerve.
Co-expression of Hsap\HSPA1LScer\UAS.cWa partially suppresses the eye degeneration seen in flies expressing Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF. Co-expression of DnaJ-1Scer\UAS.T:Zzzz\FLAG partially suppresses the eye degeneration seen in flies expressing Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF. Co-expression of both Hsap\HSPA1LScer\UAS.cWa and DnaJ-1Scer\UAS.T:Zzzz\FLAG completely suppresses the eye degeneration seen in flies expressing Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF.
The eye neurodegeneration phenotype of Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1; Scer\GAL4GMR.PF flies is not supressed by pucScer\UAS.cMa.
Co-expression of DnaJ-1[Scer\UAS.T:Zzzz\FLAG] restores external eye structure in animals expressing Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU]. Internal eye structure is partially rescued, with partial preservation of photoreceptor rhabdomere specialisations. Co-expression of DnaJ-1[Scer\UAS.T:Zzzz\FLAG] slow the progressive retinal degeneration seen in flies that weakly express Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU]. Co-expression of either DnaJ-1[ΔJ.Scer\UAS.T:Zzzz\FLAG] or DnaJ-1[G295D.Scer\UAS.T:Zzzz\FLAG] enhances the weak eye phenotype seen in flies that weakly express Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU], such that loss of pigmentation and collapse of the eye is seen. Co-expression of DnaJ-H[Scer\UAS.T:Zzzz\FLAG] only weakly suppresses the loss of eye pigmentation seen in flies expressing Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU]. Expression of DnaJ-1[Scer\UAS.T:Zzzz\FLAG] cannot suppress the severe eye degeneration seen in flies co-expressing Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] and Hsc70-4[K71S.Scer\UAS] under the control of Scer\GAL4[GMR.PU]. Flies that co-express Hsap\HSPA1L[Scer\UAS.cWa], Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] and DnaJ-1[ΔJ.Scer\UAS.T:Zzzz\FLAG] under the control of Scer\GAL4[GMR.PU] have normal eyes. Co-expression of Hsap\HSPA1L[Scer\UAS.cWa] partially suppresses the retinal degeneration seen in flies expressing Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU]. Simultaneous co-expression of both DnaJ-1[Scer\UAS.T:Zzzz\FLAG] and Hsap\HSPA1L[Scer\UAS.cWa] results in a stronger suppression of the retinal degeneration caused by expression of Hsap\ATXN3[tr.Q78.Scer\UAS.T:Ivir\HA1] under the control of Scer\GAL4[GMR.PU] than expression of either construct alone.
The eye phenotype caused by expression of Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF is suppressed by coexpression of Hsap\HSPA1LScer\UAS.cWa; the eye appears normal externally, and internally eye structure is largely restored, although photoreceptor rhabdomere specialisations are not made. The eye degeneration phenotype caused by Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 expressed under the control of Scer\GAL4GMR.PF is not suppressed by coexpression of Hsap\MAPTScer\UAS.cAa or eyaScer\UAS.cBa. Adult viability in flies expressing Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4elav-C155 is partially restored by coexpression of Hsap\HSPA1LScer\UAS.cWa and degeneration of the eye is slowed. Hsc70-4K71S.Scer\UAS enhances the disruption of the eye caused by expression of Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF.
Co-expression of BacA\p35Scer\UAS.cHa in flies expressing Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1 under the control of Scer\GAL4GMR.PF partially restores eye pigmentation.
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Hsap\ATXN3tr.Q78.Scer\UAS.T:Ivir\HA1
 
Hsap\MJDQ78.Scer\UAS.T:Ivir\HA1
 
Hsap\MJDQ78.UAS.T:HA1
 
Hsap\MJDtr.Q78.Scer\UAS.T:Ivir\HA1
 
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Bhutani et al., 2012, Cell Death Dis. 3: e428
Dysregulation of core components of SCF complex in poly-glutamine disorders. [FBrf0219951]
Butler et al., 2012, PLoS Genet. 8(2): e1002488
The Mitochondrial Chaperone Protein TRAP1 Mitigates α-Synuclein Toxicity. [FBrf0217423]
Liu et al., 2012, Nature 482(7386): 519--523
The microRNA miR-34 modulates ageing and neurodegeneration in Drosophila. [FBrf0217556]
Chang et al., 2011, PLoS Genet. 7(2): e1001288
Pathogenic VCP/TER94 Alleles Are Dominant Actives and Contribute to Neurodegeneration by Altering Cellular ATP Level in a Drosophila IBMPFD Model. [FBrf0213008]
Park et al., 2011, Biochem. Biophys. Res. Commun. 404(2): 638--645
Normal prion protein in Drosophila enhances the toxicity of pathogenic polyglutamine proteins and alters susceptibility to oxidative and autophagy signaling modulators. [FBrf0212784]
Tonoki et al., 2011, Genes Cells 16(5): 557--564
Aging causes distinct characteristics of polyglutamine amyloids in vivo. [FBrf0213535]
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