Zebrafish models for human FKRP muscular dystrophies - Wikidata - awgldk - TriplyDB

Zebrafish models for human FKRP muscular dystrophies

Zebrafish models for human FKRP muscular dystrophies

http://www.wikidata.org/entity/Q33594227

about

Fam65b is important for formation of the HDAC6-dysferlin protein complex during myogenic cell differentiation Swimming into prominence: the zebrafish as a valuable tool for studying human myopathies and muscular dystrophies.Insights from molecular dynamics simulations: structural basis for the V567D mutation-induced instability of zebrafish alpha-dystroglycan and comparison with the murine model Characterization of Zebrafish Models of Marinesco-Sjögren Syndrome In vivo modeling of the morbid human genome using Danio rerio The zebrafish dag1 mutant: a novel genetic model for dystroglycanopathies.POMK mutations disrupt muscle development leading to a spectrum of neuromuscular presentations Drug screening in a zebrafish model of Duchenne muscular dystrophy.Micromechanical function of myofibrils isolated from skeletal and cardiac muscles of the zebrafish.Zebrafish based small molecule screens for novel DMD drugs.Post-Natal knockdown of fukutin-related protein expression in muscle by long-termRNA interference induces dystrophic pathology [corrected]Zebrafish Fukutin family proteins link the unfolded protein response with dystroglycanopathies GlycoFish: a database of zebrafish N-linked glycoproteins identified using SPEG method coupled with LC/MS Mislocalization of fukutin protein by disease-causing missense mutations can be rescued with treatments directed at folding amelioration Mutations in the satellite cell gene MEGF10 cause a recessive congenital myopathy with minicores In vivo assessment of contractile strength distinguishes differential gene function in skeletal muscle of zebrafish larvae.Zebrafish models flex their muscles to shed light on muscular dystrophies.Analysis of Zebrafish Larvae Skeletal Muscle Integrity with Evans Blue Dye.Exome sequencing and functional validation in zebrafish identify GTDC2 mutations as a cause of Walker-Warburg syndrome.Expression of DUX4 in zebrafish development recapitulates facioscapulohumeral muscular dystrophy.Animal models of muscular dystrophy Force measurement during contraction to assess muscle function in zebrafish larvae.Identification of novel MYO18A interaction partners required for myoblast adhesion and muscle integrity.Neuromuscular disorders in zebrafish: state of the art and future perspectives.Mammalian O-mannosylation pathway: glycan structures, enzymes, and protein substrates.Recent advancements in understanding mammalian O-mannosylation.Tmem2 regulates cell-matrix interactions that are essential for muscle fiber attachment The psychoactive drug 25B-NBOMe recapitulates rhabdomyolysis in zebrafish larvae.The HDAC Inhibitor TSA Ameliorates a Zebrafish Model of Duchenne Muscular Dystrophy Characterization of zebrafish dysferlin by morpholino knockdown.Second international workshop for glycosylation defects in muscular dystrophies, 11-12 November, 2010, Charlotte, USA.Systems glycomics of adult zebrafish identifies organ-specific sialylation and glycosylation patterns Dose-Dependent Effects of FKRP Gene-Replacement Therapy on Functional Rescue and Longevity in Dystrophic Mice A limb-girdle muscular dystrophy 2I model of muscular dystrophy identifies corrective drug compounds for dystroglycanopathies

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P2860

Zebrafish models for human FKRP muscular dystrophies

http://www.wikidata.org/entity/Q33594227

description

2009 nî lūn-bûn

@nan

2009 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած

@hyw

2009 թվականի դեկտեմբերին հրատարակված գիտական հոդված

@hy

2009年の論文

@ja

2009年論文

@yue

2009年論文

@zh-hant

2009年論文

@zh-hk

2009年論文

@zh-mo

2009年論文

@zh-tw

2009年论文

@wuu

name

Zebrafish models for human FKRP muscular dystrophies

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Zebrafish models for human FKRP muscular dystrophies

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type

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Zebrafish models for human FKRP muscular dystrophies

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Zebrafish models for human FKRP muscular dystrophies

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Zebrafish models for human FKRP muscular dystrophies

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Zebrafish models for human FKRP muscular dystrophies

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Human Molecular Genetics

P1476

Zebrafish models for human FKRP muscular dystrophies

@en

P2093

Genri Kawahara

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

Jeffrey R Guyon

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

Louis M Kunkel

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

Yukio Nakamura

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

P2860

Muscular dystrophy and neuronal migration disorder caused by mutations in a glycosyltransferase, POMGnT1

An ancient retrotransposal insertion causes Fukuyama-type congenital muscular dystrophy

Mutations in the fukutin-related protein gene (FKRP) cause a form of congenital muscular dystrophy with secondary laminin alpha2 deficiency and abnormal glycosylation of alpha-dystroglycan.

Mutations in the O-mannosyltransferase gene POMT1 give rise to the severe neuronal migration disorder Walker-Warburg syndrome

POMT2 mutations cause alpha-dystroglycan hypoglycosylation and Walker-Warburg syndrome

The zebrafish candyfloss mutant implicates extracellular matrix adhesion failure in laminin alpha2-deficient congenital muscular dystrophy.

A stoichiometric complex of neurexins and dystroglycan in brain

Stages of embryonic development of the zebrafish

Primary structure of dystrophin-associated glycoproteins linking dystrophin to the extracellular matrix

Spectrum of brain changes in patients with congenital muscular dystrophy and FKRP gene mutations

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623-633

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scholarly article

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10.1093/HMG/DDP528

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4

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19

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2009-12-01T00:00:00Z

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19955119

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2807370

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