Absence of post-phosphoryl modification in dystroglycanopathy mouse models and wild-type tissues expressing non-laminin binding form of α-dystroglycan.
about
The glucuronyltransferase B4GAT1 is required for initiation of LARGE-mediated α-dystroglycan functional glycosylationMouse fukutin deletion impairs dystroglycan processing and recapitulates muscular dystrophyAGO61-dependent GlcNAc modification primes the formation of functional glycans on α-dystroglycanMammalian protein glycosylation--structure versus function.Adeno-associated virus-mediated overexpression of LARGE rescues α-dystroglycan function in dystrophic mice with mutations in the fukutin-related protein.Contribution of dysferlin deficiency to skeletal muscle pathology in asymptomatic and severe dystroglycanopathy models: generation of a new model for Fukuyama congenital muscular dystrophyFukutin is prerequisite to ameliorate muscular dystrophic phenotype by myofiber-selective LARGE expression.Dissecting the molecular basis of the role of the O-mannosylation pathway in disease: α-dystroglycan and forms of muscular dystrophy.Mammalian O-mannosylation pathway: glycan structures, enzymes, and protein substrates.Finding the sweet spot: assembly and glycosylation of the dystrophin-associated glycoprotein complex.Glycobiology of α-dystroglycan and muscular dystrophy.Golgi phosphoprotein 3 mediates the Golgi localization and function of protein O-linked mannose β-1,2-N-acetlyglucosaminyltransferase 1.Muscle and heart function restoration in a limb girdle muscular dystrophy 2I (LGMD2I) mouse model by systemic FKRP gene delivery.RPTPζ/phosphacan is abnormally glycosylated in a model of muscle-eye-brain disease lacking functional POMGnT1POMK mutation in a family with congenital muscular dystrophy with merosin deficiency, hypomyelination, mild hearing deficit and intellectual disability.Cystic kidneys in fetal Walker-Warburg syndrome with POMT2 mutation: Intrafamilial phenotypic variability in four siblings and review of literature.
P2860
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P2860
Absence of post-phosphoryl modification in dystroglycanopathy mouse models and wild-type tissues expressing non-laminin binding form of α-dystroglycan.
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
2012年论文
@zh
2012年论文
@zh-cn
name
Absence of post-phosphoryl mod ...... inding form of α-dystroglycan.
@ast
Absence of post-phosphoryl mod ...... inding form of α-dystroglycan.
@en
type
label
Absence of post-phosphoryl mod ...... inding form of α-dystroglycan.
@ast
Absence of post-phosphoryl mod ...... inding form of α-dystroglycan.
@en
prefLabel
Absence of post-phosphoryl mod ...... inding form of α-dystroglycan.
@ast
Absence of post-phosphoryl mod ...... inding form of α-dystroglycan.
@en
P2093
P2860
P356
P1476
Absence of post-phosphoryl mod ...... inding form of α-dystroglycan.
@en
P2093
Atsushi Kuga
Atsushi Sudo
Hiroshi Manya
Kazuhiro Kobayashi
Michiko Tajiri
Motoi Kanagawa
Motoyoshi Nomizu
Tamao Endo
Tatsushi Toda
P2860
P304
P356
10.1074/JBC.M111.271767
P407
P577
2012-01-23T00:00:00Z