The glucuronyltransferase B4GAT1 is required for initiation of LARGE-mediated α-dystroglycan functional glycosylation
about
The secretory pathway kinasesThe Structure of the T190M Mutant of Murine α-Dystroglycan at High Resolution: Insight into the Molecular Basis of a Primary DystroglycanopathyA POGLUT1 mutation causes a muscular dystrophy with reduced Notch signaling and satellite cell loss.The Muscular Dystrophy Gene TMEM5 Encodes a Ribitol β1,4-Xylosyltransferase Required for the Functional Glycosylation of Dystroglycan.The functional O-mannose glycan on α-dystroglycan contains a phospho-ribitol primed for matriglycan additionSecretome analysis identifies novel signal Peptide peptidase-like 3 (Sppl3) substrates and reveals a role of Sppl3 in multiple Golgi glycosylation pathways.Neurons and glia modify receptor protein-tyrosine phosphatase ζ (RPTPζ)/phosphacan with cell-specific O-mannosyl glycans in the developing brain.Ectopic clustering of Cajal-Retzius and subplate cells is an initial pathological feature in Pomgnt2-knockout mice, a model of dystroglycanopathy.Milder forms of muscular dystrophy associated with POMGNT2 mutations.ISPD produces CDP-ribitol used by FKTN and FKRP to transfer ribitol phosphate onto α-dystroglycan.Four-week rapamycin treatment improves muscular dystrophy in a fukutin-deficient mouse model of dystroglycanopathyLARGE2-dependent glycosylation confers laminin-binding ability on proteoglycans.Structure of protein O-mannose kinase reveals a unique active site architecture.Matriglycan: a novel polysaccharide that links dystroglycan to the basement membrane.What Have We Learned from Glycosyltransferase Knockouts in Mice?Recent advancements in understanding mammalian O-mannosylation.Differentiation-related glycan epitopes identify discrete domains of the muscle glycocalyx.The evolution of the dystroglycan complex, a major mediator of muscle integrity.Direct Mapping of Additional Modifications on Phosphorylated O-glycans of α-Dystroglycan by Mass Spectrometry Analysis in Conjunction with Knocking Out of Causative Genes for Dystroglycanopathy.Muscular Dystrophy with Ribitol-Phosphate Deficiency: A Novel Post-Translational Mechanism in Dystroglycanopathy.A dystroglycan mutation (p.Cys667Phe) associated to muscle-eye-brain disease with multicystic leucodystrophy results in ER-retention of the mutant protein.Expression in retinal neurons of fukutin and FKRP, the protein products of two dystroglycanopathy-causative genes.Rapid screening of sugar-nucleotide donor specificities of putative glycosyltransferases.A New Mouse Model of Limb-Girdle Muscular Dystrophy Type 2I Homozygous for the Common L276I Mutation Mimicking the Mild Phenotype in Humans.Overexpression of Mutant FKRP Restores Functional Glycosylation and Improves Dystrophic Phenotype in FKRP Mutant Mice.Ribitol restores functionally glycosylated α-dystroglycan and improves muscle function in dystrophic FKRP-mutant micePhosphotyrosine profiling of human cerebrospinal fluid
P2860
00baffba2050eadbfd2f5aca62f7526682512b410c63ebe4216de12e82950769a3cd148e60f324501f2429fa5f54d02bdb23f9d2128b26759dc3babe2f09fab638b124afd997fa97c5eb910bed4ad8e54283d88d25c47aecc259016324e3a843c980d2045fd95fb559efbb832c54b6f8c532cb0ab82ae90172e9170d45f7682f7946512fa739e3d97530a8607519da26fee8f580c01a60b6db88a53fe1ea67938f9f925370dea11615396caf547c4adf0c0a31d89316f6f35573d231baefcaab8211d0099810776fca955faf89387af97c9b5b737b2b6cad62db8053
P248
Q28085305-3EDEB0A9-209D-4657-911D-C42EB6964FB8Q28546951-65F170E0-21D2-4009-9037-D1A0C9421FEEQ29347528-E32D7D06-1EB2-466E-90FF-79ACB1C2294EQ32884430-F9A4B92E-9971-454E-812E-235D5203B999Q32884479-228F4B07-8F08-4F12-AA8D-0FEB7F361445Q34469573-DD29FD91-BACE-4594-978F-D602D98E687EQ35451121-4772319C-F664-49FC-8FB8-4F31A9D6FAC7Q35709549-874B2A3C-F676-49EF-9CA1-220CB35DB93BQ36738591-FACC8AFD-B906-4887-A4FB-14FE9532FC51Q36919737-5F6EF1BE-EF26-418C-85A9-811F69A993EDQ36960788-7241B556-3D63-4F97-9CC4-B69CB3517C8BQ37472546-A5899297-2409-4A2D-B789-E51F77F0E8E5Q37482125-D4BA5439-7C23-4A24-B7AE-D90DE90D1537Q38424785-7F9979A5-BF71-4078-8E14-2C37EA7F0396Q38796983-F73C0FE0-13E9-4577-AFE9-1322C56A2636Q40092479-951C39BF-428E-4DDB-9013-E535FB384A5AQ41642554-13FE2A38-FE67-4E30-AD22-4C8DAF77D6E2Q41820882-0A395559-ECB1-4198-81F6-079FE178F154Q42687793-E47552D4-4C92-4AC4-8AA5-C396983A9FC5Q47168266-74301A16-C837-47CD-877B-0DC63A9CB84FQ47405948-8CB35CB6-3DA3-475A-88D9-C5A0664EDF59Q49685437-DDD8237C-A12C-4ABF-84AC-C1326AA2DAF5Q50325365-5125CE55-1995-49CD-BCFA-EEF312CF6647Q50911860-A70E302C-8C5A-4B8D-AFE5-80CBD63E0126Q55440689-CE427204-AD8D-4817-97E3-FBF18AD9310AQ58701413-1E9D5D36-0A52-4997-938E-81A2812BAC00Q58747150-3EF126AD-55BC-4F9D-944F-D7F2363BE6DA
P2860
The glucuronyltransferase B4GAT1 is required for initiation of LARGE-mediated α-dystroglycan functional glycosylation
description
2014 nî lūn-bûn
@nan
2014 թուականին հրատարակուած գիտական յօդուած
@hyw
2014 թվականին հրատարակված գիտական հոդված
@hy
2014年の論文
@ja
2014年論文
@yue
2014年論文
@zh-hant
2014年論文
@zh-hk
2014年論文
@zh-mo
2014年論文
@zh-tw
2014年论文
@wuu
name
The glucuronyltransferase B4GA ...... lycan functional glycosylation
@ast
The glucuronyltransferase B4GA ...... lycan functional glycosylation
@en
The glucuronyltransferase B4GA ...... lycan functional glycosylation
@en-gb
The glucuronyltransferase B4GA ...... lycan functional glycosylation
@nl
type
label
The glucuronyltransferase B4GA ...... lycan functional glycosylation
@ast
The glucuronyltransferase B4GA ...... lycan functional glycosylation
@en
The glucuronyltransferase B4GA ...... lycan functional glycosylation
@en-gb
The glucuronyltransferase B4GA ...... lycan functional glycosylation
@nl
prefLabel
The glucuronyltransferase B4GA ...... lycan functional glycosylation
@ast
The glucuronyltransferase B4GA ...... lycan functional glycosylation
@en
The glucuronyltransferase B4GA ...... lycan functional glycosylation
@en-gb
The glucuronyltransferase B4GA ...... lycan functional glycosylation
@nl
P2093
P2860
P921
P3181
P356
P1433
P1476
The glucuronyltransferase B4GA ...... lycan functional glycosylation
@en
P2093
Corinne Harvey
Daniel Beltrán Valero de Bernabé
David Venzke
Greg Morgensen
Kei-Ichiro Inamori
Kevin M Wright
Tobias Willer
P2860
P3181
P356
10.7554/ELIFE.03941
P407
P577
2014-10-03T00:00:00Z