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Published in 2019 at "Advanced Science"
DOI: 10.1002/advs.201801982
Abstract: Abstract Collagen is the most abundant structural protein in mammals and is crucial for the mechanical integrity of tissues. Hsp47, an endoplasmic reticulum resident collagenāspecific chaperone, is involved in collagen biosynthesis and plays a fundamental…
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Keywords:
collagen;
secretion;
hsp47 tool;
hsp47 ... See more keywords
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Published in 2020 at "Expert opinion on therapeutic targets"
DOI: 10.1080/14728222.2021.1861249
Abstract: Introduction: Chronic fibrotic disorders are challenging clinical problems. The major challenge is the identification of specific targets expressed selectively in fibrotic tissues to avoid potential side effects of inhibiting ubiquitous factors in healthy tissues. Extensive…
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Keywords:
collagen;
fibrotic diseases;
fibrosis;
target ... See more keywords
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Published in 2018 at "Blood"
DOI: 10.1182/blood-2017-04-779934
Abstract: Chronic graft-versus-host disease (GVHD) after allogeneic hematopoietic stem cell transplantation (SCT) is characterized by multiorgan fibrosis and profoundly affects the quality of life of transplant survivors. Heat shock protein 47 (HSP47), a collagen-specific molecular chaperone,…
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Keywords:
fibrosis;
versus host;
fibrosis chronic;
hsp47 ... See more keywords
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Published in 2021 at "PLoS genetics"
DOI: 10.1371/journal.pgen.1009339
Abstract: Heat shock protein 47 (HSP47), encoded by the SERPINH1 gene, is a molecular chaperone essential for correct folding of collagens. We report a homozygous p.(R222S) substitution in HSP47 in a child with severe osteogenesis imperfecta…
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Keywords:
collagen;
osteogenesis imperfecta;
hsp47;
type collagen ... See more keywords