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Cellular stress due to impairment of collagen prolyl hydroxylation complex is rescued by the chapero...

Cellular stress due to impairment of collagen prolyl hydroxylation complex is rescued by the chapero...

https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_doaj_primary_oai_doaj_org_article_9f97c041deb94e468d25a67f4be54661

Cellular stress due to impairment of collagen prolyl hydroxylation complex is rescued by the chaperone 4-phenylbutyrate

About this item

Full title

Cellular stress due to impairment of collagen prolyl hydroxylation complex is rescued by the chaperone 4-phenylbutyrate

Publisher

England: The Company of Biologists Ltd

Journal title

Disease models & mechanisms, 2019-06, Vol.12 (6)

Language

English

Formats

Publication information

Publisher

England: The Company of Biologists Ltd

More information

Scope and Contents

Contents

Osteogenesis imperfecta (OI) types VII, VIII and IX, caused by recessive mutations in cartilage-associated protein (
), prolyl-3-hydroxylase 1 (
) and cyclophilin B (
), respectively, are characterized by the synthesis of overmodified collagen. The genes encode for the components of the endoplasmic reticulum (ER) complex responsible for th...

Alternative Titles

Full title

Cellular stress due to impairment of collagen prolyl hydroxylation complex is rescued by the chaperone 4-phenylbutyrate

Identifiers

Primary Identifiers

Record Identifier

TN_cdi_doaj_primary_oai_doaj_org_article_9f97c041deb94e468d25a67f4be54661

Permalink

https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_doaj_primary_oai_doaj_org_article_9f97c041deb94e468d25a67f4be54661

Other Identifiers

ISSN

1754-8403

E-ISSN

1754-8411

DOI

10.1242/dmm.038521

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