Articles with "p23h" as a keyword



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Phenotypic characterization of P23H and S334ter rhodopsin transgenic rat models of inherited retinal degeneration

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Published in 2018 at "Experimental Eye Research"

DOI: 10.1016/j.exer.2017.10.023

Abstract: Abstract We produced 8 lines of transgenic (Tg) rats expressing one of two different rhodopsin mutations in albino Sprague‐Dawley (SD) rats. Three lines were generated with a proline to histidine substitution at codon 23 (P23H),… read more here.

Keywords: p23h; rhodopsin; s334ter; degeneration ... See more keywords
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Lysosome docking to WIPI1 rings and ER-connected phagophores occurs during DNAJB12- and GABARAP-dependent selective autophagy of misfolded P23H-rhodopsin

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Published in 2022 at "Molecular Biology of the Cell"

DOI: 10.1091/mbc.e21-10-0505

Abstract: We report on how the endoplasmic reticulum (ER)-associated-autophagy pathway (ERAA) delivers P23H-rhodopsin (P23H-R) to the lysosome. P23H-R accumulates in an ERAD-resistant conformation that is stabilized in a detergent-soluble state by DNAJB12 and Hsp70. P23H-R, DNAJB12,… read more here.

Keywords: p23h; p23h rhodopsin; connected phagophores; gabarap ... See more keywords