Folding and assembly of type X collagen mutants that cause metaphyseal chondrodysplasia-type schmid. Evidence for co-assembly of the mutant and wild-type chains and binding to molecular chaperones.
about
Self-assembly and supramolecular organization of EMILINA dominant interference collagen X mutation disrupts hypertrophic chondrocyte pericellular matrix and glycosaminoglycan and proteoglycan distribution in transgenic miceCollagens and collagen-related diseases.COL10A1 nonsense and frame-shift mutations have a gain-of-function effect on the growth plate in human and mouse metaphyseal chondrodysplasia type Schmid.Chicken collagen X regulatory sequences restrict transgene expression to hypertrophic cartilage in mice.Growth plate compressions and altered hematopoiesis in collagen X null mice.Hypertrophic chondrocytes have a limited capacity to cope with increases in endoplasmic reticulum stress without triggering the unfolded protein responseCollagen X chains harboring Schmid metaphyseal chondrodysplasia NC1 domain mutations are selectively retained and degraded in stably transfected cells.Misfolding of collagen X chains harboring Schmid metaphyseal chondrodysplasia mutations results in aberrant disulfide bond formation, intracellular retention, and activation of the unfolded protein response.
P2860
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P2860
Folding and assembly of type X collagen mutants that cause metaphyseal chondrodysplasia-type schmid. Evidence for co-assembly of the mutant and wild-type chains and binding to molecular chaperones.
description
1999 nî lūn-bûn
@nan
1999年の論文
@ja
1999年学术文章
@wuu
1999年学术文章
@zh-cn
1999年学术文章
@zh-hans
1999年学术文章
@zh-my
1999年学术文章
@zh-sg
1999年學術文章
@yue
1999年學術文章
@zh
1999年學術文章
@zh-hant
name
Folding and assembly of type X ...... nding to molecular chaperones.
@en
type
label
Folding and assembly of type X ...... nding to molecular chaperones.
@en
prefLabel
Folding and assembly of type X ...... nding to molecular chaperones.
@en
P2860
P356
P1476
Folding and assembly of type X ...... inding to molecular chaperones
@en
P2093
P2860
P304
P356
10.1074/JBC.274.11.7570
P407
P577
1999-03-01T00:00:00Z