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. 2007 Oct 19;3(10):e177. doi: 10.1371/journal.pgen.0030177

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© 2007 Bilen and Bonini.

This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are properly credited.

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(A) Control with normal eye and retinal thickness (white arrow). Genotype w; gmr-GAL4/+.

(B) Expression of strong SCA3trQ78 causes external and internal degeneration, with pigment loss and severely reduced retinal thickness. Genotype w; gmr-GAL4 UAS-SCA3trQ78/+.

(C–E) Class 1 suppressors Hsp68^E407, mrj^E1050, and CG14207^EP1348 rescued external eye pigmentation and restored the internal eye towards normal. Genotypes w; gmr-GAL4 UAS-SCA3trQ78 in trans to alleles indicated.

(F and G) Class 3 suppressors emb^E128-1A and dbr^EP9 have strong and weak rescue. Genotypes w; gmr-GAL4 UAS-SCA3trQ78 in trans to alleles indicated.

(H–J) Co-expression of CG11033^EP3093 with SCA3trQ61 severely enhances pigment loss and internal retinal degeneration; expression of CG11033^EP3093 alone has no effect. Genotypes (H) w; gmr-GAL4 UAS-SCA3trQ61/+, (I) w; gmr-GAL4 UAS-SCA3trQ61 in trans to CG11033^EP3093, and (J) w; gmr-GAL4 in trans to CG11033^EP3093.

(K–O) Deletions for regions that uncover select modifier genes enhance Ataxin-3 degeneration, suggesting dose sensitivity of these modifiers. (K) Pathogenic Ataxin-3 causes neuronal degeneration. (L) Ataxin-3 in trans to Df(3L)CH20, which uncovers both DnaJ-1 and Ubp64E, is lethal and causes severe degeneration (pupal eye shown). (M–O) Ataxin-3 in trans to deficiencies uncovering Tpr2 (Df(2L)r10), emb (Df(2L)TE29A), or dbr (Df(2L)PM44). Genotypes (K) w; gmr-GAL4 UAS-SCA3trQ78/+, (L) w; gmr-GAL4 UAS-SCA3trQ78 /+; Df(3L)CH10/+, (M) w;gmr-GAL4 UAS-SCA3trQ78/ Df(2L)r10, (N) w;gmr-GAL4 UAS-SCA3trQ78/ Df(2L)TE29A, and (O) w;gmr-GAL4 UAS-SCA3trQ78/ Df(2L)PM44.

(P and Q) Modifiers functionally restore vision. (P) Flies with normal vision chose light when given a choice between light and dark chambers; flies expressing SCA3tr-Q78 are blind, choosing light and dark in equal numbers (1-d flies). Co-expression of mrj^E1050 or CG14207^EP1348 with SCA3trQ78 restored vision toward normal. Genotypes: Canton-S, w; gmr-GAL4 UAS-SCA3trQ78 in trans to +, mrj^E1050 or CG14207^EP1348.

(Q) Modifiers mitigate vision loss induced by full-length pathogenic Ataxin-3. Flies expressing SCA3-Q84 have strikingly compromised vision (7-d flies). Coexpression of CG14207^EP1348 or mrj^E1050 suppressed visual defects and restored phototaxis. Mean ± standard error of the mean of three experiments. Genotypes w; gmr-GAL4 UAS-SCA3Q84 in trans to +, mrj^E1050 or CG14207^EP1348.

Bar in (A), 100 μm for (A–J); bar in (K), 100 μm for (K–O).