Roles of the endoplasmic reticulum–resident, collagen-specific molecular chaperone Hsp47 in vertebrate cells and human disease

Ito, Shinya; Nagata, Kazuhiro

doi:10.1074/jbc.tm118.002812

Public

Roles of the endoplasmic reticulum–resident, collagen-specific molecular chaperone Hsp47 in vertebrate cells and human disease

Published in Journal of Biological Chemistry • Dec 12, 2018

Authors:

Shinya Ito

Kazuhiro Nagata

Abstract

gene in mice causes embryonic lethality due to impaired basement membrane and collagen fibril formation. Increased Hsp47 expression is associated with collagen-related disorders such as fibrosis, characterized by abnormal collagen accumulation, highlighting Hsp47's potential as a clinically relevant...

Subject

Procollagen peptidase

Endoplasmic reticulum

Serpin

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