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

Heat shock protein 47 (Hsp47) is an endoplasmic reticulum (ER)-resident molecular chaperone essential for correct folding of procollagen in mammalian cells. In this Review, we discuss the role and function of Hsp47 in vertebrate cells and its role in connective tissue disorders. Hsp47 binds to colla...

Subject

Procollagen peptidase

Endoplasmic reticulum

Serpin

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