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

Mutations in the human laminin alpha2 (LAMA2) gene result in the most common form of congenital muscular dystrophy (MDC1A). There are currently three models for the molecular basis of cellular pathology in MDC1A: (i) lack of LAMA2 leads to sarcolemmal weakness and failure, followed by cellular necro...

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Bibliographic Details
Published in:	Proceedings of the National Academy of Sciences - PNAS Vol. 104; no. 17; p. 7092
Main Authors:	Hall, Thomas E, Bryson-Richardson, Robert J, Berger, Silke, Jacoby, Arie S, Cole, Nicholas J, Hollway, Georgina E, Berger, Joachim, Currie, Peter D
Format:	Journal Article
Language:	English
Published:	United States 24.04.2007
Subjects:	Adhesiveness - drug effects Alleles Amino Acid Sequence Animals Base Sequence Cell Death - drug effects Codon, Nonsense - genetics Embryo, Nonmammalian - cytology Embryo, Nonmammalian - drug effects Embryo, Nonmammalian - innervation Embryo, Nonmammalian - ultrastructure Extracellular Matrix - drug effects Extracellular Matrix - metabolism Gene Expression Regulation - drug effects Intercellular Junctions - drug effects Intercellular Junctions - ultrastructure Laminin - chemistry Laminin - deficiency Laminin - genetics Laminin - metabolism Molecular Sequence Data Motor Activity - drug effects Muscle Fibers, Skeletal - drug effects Muscle Fibers, Skeletal - pathology Muscular Dystrophy, Animal - congenital Mutant Proteins - metabolism Oligonucleotides, Antisense - pharmacology Open Reading Frames - genetics Sarcolemma - drug effects Sarcolemma - pathology Sequence Homology, Amino Acid Zebrafish - abnormalities Zebrafish - embryology Zebrafish Proteins - metabolism
ISSN:	0027-8424
Online Access:	Get more information
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