Mutations in the ER-Golgi intermediate compartment protein ERGIC-53 cause combined deficiency of coagulation factors V and VIII
about
Sec24 proteins and sorting at the endoplasmic reticulumLocalization of alpha 1,3-fucosyltransferase VI in Weibel-Palade bodies of human endothelial cellsSequential steps and checkpoints in the early exocytic compartment during secretory IgM biogenesisProfile-based data base scanning for animal L-type lectins and characterization of VIPL, a novel VIP36-like endoplasmic reticulum proteinProteomics of endoplasmic reticulum-Golgi intermediate compartment (ERGIC) membranes from brefeldin A-treated HepG2 cells identifies ERGIC-32, a new cycling protein that interacts with human Erv46COG-7-deficient Human Fibroblasts Exhibit Altered Recycling of Golgi ProteinsThe cargo receptors Surf4, endoplasmic reticulum-Golgi intermediate compartment (ERGIC)-53, and p25 are required to maintain the architecture of ERGIC and Golgi.Assembly, organization, and function of the COPII coatCombined deficiency of coagulation factors V and VIII: an updateN-linked sugar-regulated protein folding and quality control in the ERCrystal structure of the carbohydrate recognition domain of p58/ERGIC-53, a protein involved in glycoprotein export from the endoplasmic reticulumCrystal structure of the LMAN1-CRD/MCFD2 transport receptor complex provides insight into combined deficiency of factor V and factor VIIIStructural Characterization of Carbohydrate Binding by LMAN1 Protein Provides New Insight into the Endoplasmic Reticulum Export of Factors V (FV) and VIII (FVIII)Erv26p directs pro-alkaline phosphatase into endoplasmic reticulum-derived coat protein complex II transport vesicles.Structures of the carbohydrate recognition domain of Ca2+-independent cargo receptors Emp46p and Emp47p.Efficient IgM assembly and secretion require the plasma cell induced endoplasmic reticulum protein pERp1Mutations in the MCFD2 gene are predominant among patients with hereditary combined FV and FVIII deficiency (F5F8D) in IndiaMice deficient in LMAN1 exhibit FV and FVIII deficiencies and liver accumulation of α1-antitrypsinMammalian COPII coat component SEC24C is required for embryonic development in miceLectins and traffic in the secretory pathwayThe ordered and compartment-specfific autoproteolytic removal of the furin intramolecular chaperone is required for enzyme activation.SEC24A deficiency lowers plasma cholesterol through reduced PCSK9 secretion.Quantitative ER <--> Golgi transport kinetics and protein separation upon Golgi exit revealed by vesicular integral membrane protein 36 dynamics in live cells.Identification of ERGIC-53 as an intracellular transport receptor of alpha1-antitrypsin.Endoplasmic reticulum protein targeting of phospholamban: a common role for an N-terminal di-arginine motif in ER retention?EF-hand domains of MCFD2 mediate interactions with both LMAN1 and coagulation factor V or VIII.From the ER to the golgi: insights from the study of combined factors V and VIII deficiency.Murine coagulation factor VIII is synthesized in endothelial cells.Deletion of the fibrinogen [correction of fibrogen] alpha-chain gene (FGA) causes congenital afibrogenemia.The lectin ERGIC-53 is a cargo transport receptor for glycoproteins.Carbohydrate- and conformation-dependent cargo capture for ER-exitAdvancements in gene transfer-based therapy for hemophilia A.Traffic jam: a compendium of human diseases that affect intracellular transport processes.Control of MHC class I traffic from the endoplasmic reticulum by cellular chaperones and viral anti-chaperones.Proteomic identification of S-nitrosylated Golgi proteins: new insights into endothelial cell regulation by eNOS-derived NO.Heat shock induces preferential translation of ERGIC-53 and affects its recycling pathway.The intrinsic and extrinsic effects of N-linked glycans on glycoproteostasis.Autosomal recessive deficiencies of coagulation factors.Molecular basis of LMAN1 in coordinating LMAN1-MCFD2 cargo receptor formation and ER-to-Golgi transport of FV/FVIIIRole of Sec24 isoforms in selective export of membrane proteins from the endoplasmic reticulum
P2860
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P2860
Mutations in the ER-Golgi intermediate compartment protein ERGIC-53 cause combined deficiency of coagulation factors V and VIII
description
1998 nî lūn-bûn
@nan
1998 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
1998 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
1998年の論文
@ja
1998年論文
@yue
1998年論文
@zh-hant
1998年論文
@zh-hk
1998年論文
@zh-mo
1998年論文
@zh-tw
1998年论文
@wuu
name
Mutations in the ER-Golgi inte ...... coagulation factors V and VIII
@ast
Mutations in the ER-Golgi inte ...... coagulation factors V and VIII
@en
Mutations in the ER-Golgi inte ...... coagulation factors V and VIII
@en-gb
Mutations in the ER-Golgi inte ...... coagulation factors V and VIII
@nl
type
label
Mutations in the ER-Golgi inte ...... coagulation factors V and VIII
@ast
Mutations in the ER-Golgi inte ...... coagulation factors V and VIII
@en
Mutations in the ER-Golgi inte ...... coagulation factors V and VIII
@en-gb
Mutations in the ER-Golgi inte ...... coagulation factors V and VIII
@nl
prefLabel
Mutations in the ER-Golgi inte ...... coagulation factors V and VIII
@ast
Mutations in the ER-Golgi inte ...... coagulation factors V and VIII
@en
Mutations in the ER-Golgi inte ...... coagulation factors V and VIII
@en-gb
Mutations in the ER-Golgi inte ...... coagulation factors V and VIII
@nl
P2093
P3181
P1433
P1476
Mutations in the ER-Golgi inte ...... coagulation factors V and VIII
@en
P2093
C E Hertel
D Ginsburg
M A Wheatley
M J Moussalli
N Ciavarella
R J Kaufman
U Seligsohn
P3181
P356
10.1016/S0092-8674(00)81146-0
P407
P577
1998-04-03T00:00:00Z