Intrinsic protein-protein interaction-mediated and chaperonin-assisted sequential assembly of stable bardet-biedl syndrome protein complex, the BBSome
about
Bardet-Biedl syndrome proteins 1 and 3 regulate the ciliary trafficking of polycystic kidney disease 1 proteinHuman TRiC complex purified from HeLa cells contains all eight CCT subunits and is active in vitroThe novel centriolar satellite protein SSX2IP targets Cep290 to the ciliary transition zoneBBS mutations modify phenotypic expression of CEP290-related ciliopathiesThe centriolar satellite protein AZI1 interacts with BBS4 and regulates ciliary trafficking of the BBSomeCEP290 alleles in mice disrupt tissue-specific cilia biogenesis and recapitulate features of syndromic ciliopathiesCP110 and its network of partners coordinately regulate cilia assembly.Bardet-Biedl syndrome: Genetics, molecular pathophysiology, and disease managementA novel H395R mutation in MKKS/BBS6 causes retinitis pigmentosa and polydactyly without other findings of Bardet-Biedl or McKusick-Kaufman syndromeNephrocystin proteins NPHP5 and Cep290 regulate BBSome integrity, ciliary trafficking and cargo deliveryCentriolar satellites: key mediators of centrosome functionsBardet-Biedl syndrome proteins control the cilia length through regulation of actin polymerizationPharmacological modulation of the retinal unfolded protein response in Bardet-Biedl syndrome reduces apoptosis and preserves light detection abilityDisordered proteinaceous machinesFunctional characterization of Prickle2 and BBS7 identify overlapping phenotypes yet distinct mechanisms.Direct evidence for BBSome-associated intraflagellar transport reveals distinct properties of native mammalian cilia.Essential role of the chaperonin CCT in rod outer segment biogenesisWhole Exome Sequencing Identifies a Novel and a Recurrent Mutation in BBS2 Gene in a Family with Bardet-Biedl SyndromeRegulation of Insulin Receptor Trafficking by Bardet Biedl Syndrome Proteins.Developmental disruptions underlying brain abnormalities in ciliopathies.BBS4 and BBS5 show functional redundancy in the BBSome to regulate the degradative sorting of ciliary sensory receptors.The BBSome Controls Energy Homeostasis by Mediating the Transport of the Leptin Receptor to the Plasma Membrane.The centriolar satellite proteins Cep72 and Cep290 interact and are required for recruitment of BBS proteins to the cilium.Loss of the BBSome perturbs endocytic trafficking and disrupts virulence of Trypanosoma brucei.Exome sequencing identifies a novel and a recurrent BBS1 mutation in Pakistani families with Bardet-Biedl syndromeBBS7 is required for BBSome formation and its absence in mice results in Bardet-Biedl syndrome phenotypes and selective abnormalities in membrane protein trafficking.Human CCT4 and CCT5 chaperonin subunits expressed in Escherichia coli form biologically active homo-oligomers.The ciliopathy-associated CPLANE proteins direct basal body recruitment of intraflagellar transport machinery.Light-dependent phosphorylation of Bardet-Biedl syndrome 5 in photoreceptor cells modulates its interaction with arrestin1The Leishmania major BBSome subunit BBS1 is essential for parasite virulence in the mammalian host.Exome sequencing of Bardet-Biedl syndrome patient identifies a null mutation in the BBSome subunit BBIP1 (BBS18)Genetic variations and diseases in UniProtKB/Swiss-Prot: the ins and outs of expert manual curation.Computational genomic analysis of PARK7 interactome reveals high BBS1 gene expression as a prognostic factor favoring survival in malignant pleural mesothelioma.The multifaceted roles of intrinsic disorder in protein complexes.Bardet-Biedl syndrome: Is it only cilia dysfunction?BBS4 regulates the expression and secretion of FSTL1, a protein that participates in ciliogenesis and the differentiation of 3T3-L1.Functional analysis by minigene assay of putative splicing variants found in Bardet-Biedl syndrome patients.The cilium: a cellular antenna with an influence on obesity risk.Structural and molecular bases of rod photoreceptor morphogenesis and disease.Gene Therapeutic Reversal of Peripheral Olfactory Impairment in Bardet-Biedl Syndrome.
P2860
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P2860
Intrinsic protein-protein interaction-mediated and chaperonin-assisted sequential assembly of stable bardet-biedl syndrome protein complex, the BBSome
description
2012 nî lūn-bûn
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2012 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
2012 թվականի հունիսին հրատարակված գիտական հոդված
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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
@zh-hant
2012年論文
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2012年論文
@zh-mo
2012年論文
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2012年论文
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name
Intrinsic protein-protein inte ...... me protein complex, the BBSome
@ast
Intrinsic protein-protein inte ...... me protein complex, the BBSome
@en
Intrinsic protein-protein inte ...... me protein complex, the BBSome
@en-gb
Intrinsic protein-protein inte ...... me protein complex, the BBSome
@nl
type
label
Intrinsic protein-protein inte ...... me protein complex, the BBSome
@ast
Intrinsic protein-protein inte ...... me protein complex, the BBSome
@en
Intrinsic protein-protein inte ...... me protein complex, the BBSome
@en-gb
Intrinsic protein-protein inte ...... me protein complex, the BBSome
@nl
prefLabel
Intrinsic protein-protein inte ...... me protein complex, the BBSome
@ast
Intrinsic protein-protein inte ...... me protein complex, the BBSome
@en
Intrinsic protein-protein inte ...... me protein complex, the BBSome
@en-gb
Intrinsic protein-protein inte ...... me protein complex, the BBSome
@nl
P2093
P2860
P921
P3181
P356
P1476
Intrinsic protein-protein inte ...... e protein complex, the BBSome.
@en
P2093
Qihong Zhang
Val C Sheffield
P2860
P304
P3181
P356
10.1074/JBC.M112.341487
P407
P577
2012-06-08T00:00:00Z