Reference Report

Reference
Citation	Takeyama, K., Ito, S., Yamamoto, A., Tanimoto, H., Furutani, T., Kanuka, H., Miura, M., Tabata, T., Kato, S. (2002). Androgen-dependent neurodegeneration by polyglutamine-expanded human androgen receptor in Drosophila. Neuron 35(5): 855--864. (Export to RIS)
FlyBase ID	FBrf0152267
Publication Type	Research paper
PubMed ID	12372281
PubMed Abstract	Spinal and bulbar muscular atrophy (SBMA) is an X-linked, adult-onset, neurodegenerative disorder affecting only males and is caused by expanded polyglutamine (polyQ) stretches in the N-terminal A/B domain of human androgen receptor (hAR). Although no overt phenotype was detected in adult fly eye photoreceptor neurons expressing mutant hAR (polyQ 52), ingestion of androgen or its known antagonists caused marked neurodegeneration with nuclear localization and structural alteration of the hAR mutant. Ligand-independent toxicity was detected with a truncated polyQ-expanded A/B domain alone, which was attenuated with cytosolic trapping by coexpression of the unliganded hAR E/F ligand binding domain. Thus, our findings suggest that the full binding of androgen to the polyQ-expanded hAR mutants leads to structural alteration with nuclear translocation that eventually results in the onset of SBMA in male patients.
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Language of Publication	English
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Publication Type	Journal
Abbreviation	Neuron
Title	Neuron
Publication Year	1988-
ISBN/ISSN	0896-6273
Data from Reference
Alleles (14)
	Avic\GFP^ARE DnaJ-1^Scer\UAS.cKa Hsap\AR^{AF-1.Scer\UAS} Hsap\AR^{AF-2.Scer\UAS} Hsap\AR^{Q52.Scer\UAS.T:Xlae\nes} Hsap\AR^Q52.Scer\UAS Hsap\AR^{Q52AF-1.Scer\UAS} Hsap\AR^{Q52AF-1.ΔNLS.Scer\UAS.T:Xlae\nes} Hsap\AR^Scer\UAS.cTa Scer\GAL4^dpp.blk1 Scer\GAL4^GMR.PY Scer\GAL4^ptc-559.1 w^+mC Zzzz\CAG^{127Q.Scer\UAS.T:Ivir\HA1}
Constructs (13)
	P{ARE-GFP.T} P{GAL4-dpp.blk1} P{GAL4-ninaE.GMR} P{GMR-GAL4.Y} P{UAS-DnaJ-1.K} P{UAS-Hsap\AR.AF-1} P{UAS-Hsap\AR.AF-2} P{UAS-Hsap\AR.Q52,T:Xlae\nes} P{UAS-Hsap\AR.Q52} P{UAS-Hsap\AR.Q52AF-1} P{UAS-Hsap\AR.Q52AF-1ΔNLS,T:Xlae\nes} P{UAS-Hsap\AR.wt} P{UAS-Zzzz\CAG.127Q}
Genes (11)
	Avic\GFP DnaJ-1 EcR Eip75B GMR Hsap\AR Scer\GAL4 T:Ivir\HA1 T:Xlae\nes w Zzzz\CAG
Insertions (1)
	P{GawB}ptc^559.1