Mutation in cyclophilin B that causes hyperelastosis cutis in American Quarter Horse does not affect peptidylprolyl cis-trans isomerase activity but shows altered cyclophilin B-protein interactions and affects collagen folding

Mutation in cyclophilin B that causes hyperelastosis cutis in American Quarter Horse does not affect peptidylprolyl cis-trans isomerase activity but shows altered cyclophilin B-protein interactions and affects collagen folding

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http://www.wikidata.org/entity/Q42033519

Q42033519

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Abnormal type I collagen post-translational modification and crosslinking in a cyclophilin B KO mouse model of recessive osteogenesis imperfecta Crystal structures of wild-type and mutated cyclophilin B that causes hyperelastosis cutis in the American quarter horse Co- and Post-Translational Protein Folding in the ER An additional function of the rough endoplasmic reticulum protein complex prolyl 3-hydroxylase 1·cartilage-associated protein·cyclophilin B: the CXXXC motif reveals disulfide isomerase activity in vitro.Skin malformations in a neonatal foal tested homozygous positive for Warmblood Fragile Foal Syndrome Post-translationally abnormal collagens of prolyl 3-hydroxylase-2 null mice offer a pathobiological mechanism for the high myopia linked to human LEPREL1 mutations.Cyclophilin-B Modulates Collagen Cross-linking by Differentially Affecting Lysine Hydroxylation in the Helical and Telopeptidyl Domains of Tendon Type I Collagen.Sc65-Null Mice Provide Evidence for a Novel Endoplasmic Reticulum Complex Regulating Collagen Lysyl Hydroxylation.Targeting the cyclophilin domain of Ran-binding protein 2 (Ranbp2) with novel small molecules to control the proteostasis of STAT3, hnRNPA2B1 and M-opsin.Mutations in FKBP10, which result in Bruck syndrome and recessive forms of osteogenesis imperfecta, inhibit the hydroxylation of telopeptide lysines in bone collagen.MBTPS2 mutations cause defective regulated intramembrane proteolysis in X-linked osteogenesis imperfecta.Absence of the ER Cation Channel TMEM38B/TRIC-B Disrupts Intracellular Calcium Homeostasis and Dysregulates Collagen Synthesis in Recessive Osteogenesis Imperfecta.Differential loss of prolyl isomerase or chaperone activity of Ran-binding protein 2 (Ranbp2) unveils distinct physiological roles of its cyclophilin domain in proteostasis.Collagen prolyl 3-hydroxylation: a major role for a minor post-translational modification?Distinct post-translational features of type I collagen are conserved in mouse and human periodontal ligament.Tensile properties in collagen-rich tissues of Quarter Horses with hereditary equine regional dermal asthenia (HERDA).A substrate preference for the rough endoplasmic reticulum resident protein FKBP22 during collagen biosynthesis.Ziploc-ing the structure 2.0: Endoplasmic reticulum-resident peptidyl prolyl isomerases show different activities toward hydroxyproline.Increased susceptibility of skin from HERDA (Hereditary Equine Regional Dermal Asthenia)-affected horses to bacterial collagenase degradation: a potential contributing factor to the clinical signs of HERDA.

P2860

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P2860

Mutation in cyclophilin B that causes hyperelastosis cutis in American Quarter Horse does not affect peptidylprolyl cis-trans isomerase activity but shows altered cyclophilin B-protein interactions and affects collagen folding

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http://www.wikidata.org/entity/Q42033519

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2012 nî lūn-bûn

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2012年の論文

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2012年論文

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2012年論文

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2012年論文

@zh-hk

2012年論文

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2012年論文

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2012年论文

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2012年论文

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2012年论文

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name

Mutation in cyclophilin B that ...... s and affects collagen folding

@en

Mutation in cyclophilin B that ...... s and affects collagen folding

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type

Item

label

Mutation in cyclophilin B that ...... s and affects collagen folding

@en

Mutation in cyclophilin B that ...... s and affects collagen folding

@nl

prefLabel

Mutation in cyclophilin B that ...... s and affects collagen folding

@en

Mutation in cyclophilin B that ...... s and affects collagen folding

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P1476

Mutation in cyclophilin B that ...... s and affects collagen folding

@en

P2093

Ann M Rashmir-Raven

http://www.w3.org/2001/XMLSchema#string

Douglas R Keene

http://www.w3.org/2001/XMLSchema#string

Elena Pokidysheva

http://www.w3.org/2001/XMLSchema#string

Hans Peter Bächinger

http://www.w3.org/2001/XMLSchema#string

Janice A Vranka

http://www.w3.org/2001/XMLSchema#string

Kazuhiro Nagata

http://www.w3.org/2001/XMLSchema#string

Keith Zientek

http://www.w3.org/2001/XMLSchema#string

Nena J Winand

http://www.w3.org/2001/XMLSchema#string

Sergei P Boudko

http://www.w3.org/2001/XMLSchema#string

Yoshihiro Ishikawa

http://www.w3.org/2001/XMLSchema#string

P2860

Human Ehlers-Danlos syndrome type VII C and bovine dermatosparaxis are caused by mutations in the procollagen I N-proteinase gene

Prolyl 3-hydroxylase 1 deficiency causes a recessive metabolic bone disorder resembling lethal/severe osteogenesis imperfecta

Prolyl 3-hydroxylase 1 null mice display abnormalities in fibrillar collagen-rich tissues such as tendons, skin, and bones

Cyclophilin B is a functional regulator of hepatitis C virus RNA polymerase

Defective glycosylation of decorin and biglycan, altered collagen structure, and abnormal phenotype of the skin fibroblasts of an Ehlers-Danlos syndrome patient carrying the novel Arg270Cys substitution in galactosyltransferase I (beta4GalT-7)

Prolyl 3-hydroxylase 1, enzyme characterization and identification of a novel family of enzymes

A subset of chaperones and folding enzymes form multiprotein complexes in endoplasmic reticulum to bind nascent proteins.

X-ray structure of a cyclophilin B/cyclosporin complex: comparison with cyclophilin A and delineation of its calcineurin-binding domain

Mice that lack thrombospondin 2 display connective tissue abnormalities that are associated with disordered collagen fibrillogenesis, an increased vascular density, and a bleeding diathesis

Structural Basis of Cyclophilin B Binding by the Calnexin/Calreticulin P-domain

P304

22253-22265

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scholarly article

P356

10.1074/JBC.M111.333336

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English

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http://www.w3.org/2001/XMLSchema#string

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287

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Kazunori Mizuno

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2012-05-03T00:00:00Z

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22556420

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P921

protein folding

P932

3381186

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Mutation in cyclophilin B that causes hyperelastosis cutis in American Quarter Horse does not affect peptidylprolyl cis-trans isomerase activity but shows altered cyclophilin B-protein interactions and affects collagen folding

about

P2860

P2860

Mutation in cyclophilin B that causes hyperelastosis cutis in American Quarter Horse does not affect peptidylprolyl cis-trans isomerase activity but shows altered cyclophilin B-protein interactions and affects collagen folding

description

name

type

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P1433

P1476

P2093

P2860

P304

P31

P356

P407

P433

P478

P50

P577

P698

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P356

P1433

P1476

P2093

P2860

P304

P31

P356

P407

P433

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P921

P932