Altered chondrocyte differentiation and extracellular matrix homeostasis in a zebrafish model for mucolipidosis II.
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The DMAP interaction domain of UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase is a substrate recognition moduleAnalysis of mucolipidosis II/III GNPTAB missense mutations identifies domains of UDP-GlcNAc:lysosomal enzyme GlcNAc-1-phosphotransferase involved in catalytic function and lysosomal enzyme recognitionSelective yolk deposition and mannose phosphorylation of lysosomal glycosidases in zebrafishA novel mouse model of a patient mucolipidosis II mutation recapitulates disease pathologyAbnormal cartilage development and altered N-glycosylation in Tmem165-deficient zebrafish mirrors the phenotypes associated with TMEM165-CDG.Exploring the elephant: histopathology in high-throughput phenotyping of mutant mice.Mechanisms of otoconia and otolith developmentExcessive activity of cathepsin K is associated with cartilage defects in a zebrafish model of mucolipidosis II.The glycan-binding properties of the cation-independent mannose 6-phosphate receptor are evolutionary conserved in vertebrates.Lysosomal dysfunction causes neurodegeneration in mucolipidosis II 'knock-in' mice.A zebrafish model of PMM2-CDG reveals altered neurogenesis and a substrate-accumulation mechanism for N-linked glycosylation deficiency.Cathepsin-Mediated Alterations in TGFß-Related Signaling Underlie Disrupted Cartilage and Bone Maturation Associated With Impaired Lysosomal Targeting."Casting" light on the role of glycosylation during embryonic development: insights from zebrafish.Modelling inborn errors of metabolism in zebrafish.Endothelial cell dysfunction in globoid cell leukodystrophy.Enzyme-specific differences in mannose phosphorylation between GlcNAc-1-phosphotransferase αβ and γ subunit deficient zebrafish support cathepsin proteases as early mediators of mucolipidosis pathology.Kinesin-1 promotes chondrocyte maintenance during skeletal morphogenesis.
P2860
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P2860
Altered chondrocyte differentiation and extracellular matrix homeostasis in a zebrafish model for mucolipidosis II.
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
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scientific article published on 15 October 2009
@en
vedecký článok
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vetenskaplig artikel
@sv
videnskabelig artikel
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vědecký článek
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name
Altered chondrocyte differenti ...... sh model for mucolipidosis II.
@en
Altered chondrocyte differenti ...... sh model for mucolipidosis II.
@nl
type
label
Altered chondrocyte differenti ...... sh model for mucolipidosis II.
@en
Altered chondrocyte differenti ...... sh model for mucolipidosis II.
@nl
prefLabel
Altered chondrocyte differenti ...... sh model for mucolipidosis II.
@en
Altered chondrocyte differenti ...... sh model for mucolipidosis II.
@nl
P2093
P2860
P1476
Altered chondrocyte differenti ...... sh model for mucolipidosis II.
@en
P2093
Christina Sias
Heather Flanagan-Steet
Richard Steet
P2860
P304
P356
10.2353/AJPATH.2009.090210
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P577
2009-10-15T00:00:00Z