Transgenic isolation of skeletal muscle and kidney defects in laminin beta2 mutant mice: implications for Pierson syndrome - Wikidata - awgldk - TriplyDB

Transgenic isolation of skeletal muscle and kidney defects in laminin beta2 mutant mice: implications for Pierson syndrome

Transgenic isolation of skeletal muscle and kidney defects in laminin beta2 mutant mice: implications for Pierson syndrome

http://www.wikidata.org/entity/Q34343200

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Skeletal muscle laminin and MDC1A: pathogenesis and treatment strategies.Developmental and pathogenic mechanisms of basement membrane assembly Discs-large homolog 1 regulates smooth muscle orientation in the mouse ureter.Integrins in kidney disease Update on the glomerular filtration barrier Defective formation of the inner limiting membrane in laminin beta2- and gamma3-null mice produces retinal dysplasia Proteinuria precedes podocyte abnormalities inLamb2-/- mice, implicating the glomerular basement membrane as an albumin barrier The zebrafish dystrophic mutant softy maintains muscle fibre viability despite basement membrane rupture and muscle detachment Protein-anchoring strategy for delivering acetylcholinesterase to the neuromuscular junction.Maintenance of glomerular filtration barrier integrity requires laminin alpha5.Transgenic expression of human LAMA5 suppresses murine Lama5 mRNA and laminin α5 protein deposition.Mutations in the human laminin beta2 (LAMB2) gene and the associated phenotypic spectrum.Functional consequences of cell type-restricted expression of laminin α5 in mouse placental labyrinth and kidney glomerular capillaries Extracellular matrix: functions in the nervous system Next-generation sequencing to dissect hereditary nephrotic syndrome in mice identifies a hypomorphic mutation in Lamb2 and models Pierson's syndrome Forced expression of laminin beta1 in podocytes prevents nephrotic syndrome in mice lacking laminin beta2, a model for Pierson syndrome Albumin-associated free fatty acids induce macropinocytosis in podocytes.RNA-binding protein IGF2BP2/IMP2 is required for laminin-β2 mRNA translation and is modulated by glucose concentration.Role of extracellular matrix proteins and their receptors in the development of the vertebrate neuromuscular junction Calcium channels link the muscle-derived synapse organizer laminin β2 to Bassoon and CAST/Erc2 to organize presynaptic active zones.Laminin β2 gene missense mutation produces endoplasmic reticulum stress in podocytes Mutations in LAMB2 causing a severe form of synaptic congenital myasthenic syndrome.Albumin contributes to kidney disease progression in Alport syndrome.Myotendinous junction defects and reduced force transmission in mice that lack alpha7 integrin and utrophin Organogenesis of the kidney glomerulus: focus on the glomerular basement membrane.Animal models of nephrotic syndrome.Synaptic basal lamina-associated congenital myasthenic syndromes.Laminin: loss-of-function studies.Mesencephalic astrocyte-derived neurotrophic factor (MANF), a new player in endoplasmic reticulum diseases: structure, biology, and therapeutic roles.Mesencephalic Astrocyte-Derived Neurotrophic Factor as a Urine Biomarker for Endoplasmic Reticulum Stress-Related Kidney Diseases.Development of kidney glomerular endothelial cells and their role in basement membrane assembly.A missense LAMB2 mutation causes congenital nephrotic syndrome by impairing laminin secretion.The C-terminal region of laminin beta chains modulates the integrin binding affinities of laminins.An inducible mouse model of podocin-mutation-related nephrotic syndrome.Pathogenicity of a Human Laminin β2 Mutation Revealed in Models of Alport Syndrome.Elevated urinary CRELD2 is associated with endoplasmic reticulum stress-mediated kidney disease.Basement Membrane Defects in Genetic Kidney Diseases.Laminin-521 Protein Therapy for Glomerular Basement Membrane and Podocyte Abnormalities in a Model of Pierson Syndrome.

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P2860

Transgenic isolation of skeletal muscle and kidney defects in laminin beta2 mutant mice: implications for Pierson syndrome

http://www.wikidata.org/entity/Q34343200

description

2006 nî lūn-bûn

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2006 թուականի Փետրուարին հրատարակուած գիտական յօդուած

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2006 թվականի փետրվարին հրատարակված գիտական հոդված

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2006年の論文

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2006年論文

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2006年論文

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2006年論文

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2006年論文

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2006年论文

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Transgenic isolation of skelet ...... lications for Pierson syndrome

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Transgenic isolation of skelet ...... lications for Pierson syndrome

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Transgenic isolation of skelet ...... lications for Pierson syndrome

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Transgenic isolation of skelet ...... lications for Pierson syndrome

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Transgenic isolation of skelet ...... lications for Pierson syndrome

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Transgenic isolation of skelet ...... lications for Pierson syndrome

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Transgenic isolation of skelet ...... lications for Pierson syndrome

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Transgenic isolation of skelet ...... lications for Pierson syndrome

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Transgenic isolation of skelet ...... lications for Pierson syndrome

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Transgenic isolation of skelet ...... lications for Pierson syndrome

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Bruce L Patton

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George Jarad

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Gloriosa Go

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Jeanette Cunningham

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Jeffrey H Miner

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P2860

Absence of basement membranes after targeting the LAMC1 gene results in embryonic lethality due to failure of endoderm differentiation

Molecular heterogeneity of basal laminae: isoforms of laminin and collagen IV at the neuromuscular junction and elsewhere

Positionally cloned gene for a novel glomerular protein--nephrin--is mutated in congenital nephrotic syndrome

Laminin expression in adult and developing retinae: evidence of two novel CNS laminins

The laminin alpha chains: expression, developmental transitions, and chromosomal locations of alpha1-5, identification of heterotrimeric laminins 8-11, and cloning of a novel alpha3 isoform

A simplified laminin nomenclature

Human laminin beta2 deficiency causes congenital nephrosis with mesangial sclerosis and distinct eye abnormalities

A laminin-like adhesive protein concentrated in the synaptic cleft of the neuromuscular junction

The renal glomerulus of mice lacking s-laminin/laminin beta 2: nephrosis despite molecular compensation by laminin beta 1

Synaptic laminin prevents glial entry into the synaptic cleft

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16452099

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1363729

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