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The molecular chaperone Hsp90α deficiency causes retinal degeneration by disrupting Golgi organization and vesicle transportation in photoreceptors.

J Mol Cell Biol. 2020 Apr 24;12(3):216-229
Yuan Wu 1 , Xiudan Zheng 1 , Yubo Ding 1 , Min Zhou 2 , Zhuang Wei 1 , Tao Liu 1 , Kan Liao 1
Yuan Wu 1 , Xiudan Zheng 1 , Yubo Ding 1 , Min Zhou 2 , Zhuang Wei 1 , Tao Liu 1 , Kan Liao 1
+ et al

[No authors listed]

Author information
  • 1 Key Laboratory of Systems Biology, CAS Center for Excellence in Molecular Cell Science, Shanghai Institute of Biochemistry and Cell Biology, Chinese Academy of Sciences, University of Chinese Academy of Sciences, Shanghai 200031, China.
  • 2 Department of Ophthalmology, Eye and ENT Hospital of Fudan University, Shanghai 200031, China.

摘要


Heat shock protein 90 (Hsp90) is an abundant molecular chaperone with two isoforms, Hsp90α and Hsp90β. Hsp90β deficiency causes embryonic lethality, whereas Hsp90α deficiency causes few abnormities except male sterility. In this paper, we reported that Hsp90α was exclusively expressed in the retina, testis, and brain. Its deficiency caused retinitis pigmentosa (RP), a disease leading to blindness. In Hsp90α-deficient mice, the retina was deteriorated and the outer segment of photoreceptor was deformed. Immunofluorescence staining and electron microscopic analysis revealed disintegrated Golgi and aberrant intersegmental vesicle transportation in Hsp90α-deficient photoreceptors. Proteomic analysis identified microtubule-associated protein 1B (MAP1B) as an Hsp90α-associated protein in photoreceptors. Hspα deficiency increased degradation of MAP1B by inducing its ubiquitination, causing α-tubulin deacetylation and microtubule destabilization. Furthermore, the treatment of wild-type mice with 17-DMAG, an Hsp90 inhibitor of geldanamycin derivative, induced the same retinal degeneration as Hsp90α deficiency. Taken together, the microtubule destabilization could be the underlying reason for Hsp90α deficiency-induced RP.

KEYWORDS: Golgi disintegration, Hsp90α, MAP1B, acetylated α-tubulin, cytoskeleton, microtubule, retinitis pigmentosa, vesicle transportation