Loss of basement membrane, receptor and cytoskeletal lattices in a laminin-deficient muscular dystrophy

Peter D Yurchenco; Yi-Shan Cheng; Kevin Campbell; Shaohua Li

doi:10.1242/jcs.00911

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Loss of basement membrane, receptor and cytoskeletal lattices in a laminin-deficient muscular dystrophy

Journal article

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Loss of basement membrane, receptor and cytoskeletal lattices in a laminin-deficient muscular dystrophy

Peter D Yurchenco, Yi-Shan Cheng, Kevin Campbell and Shaohua Li

Journal of cell science, Vol.117(Pt 5), pp.735-742

02/15/2004

DOI: 10.1242/jcs.00911

PMID: 14734655

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Abstract

Basement membrane laminins bearing the alpha2-subunit interact with alpha-dystroglycan and beta1-integrins, cell-surface receptors that are found within the rectilinear costameric lattices of skeletal muscle sarcolemma. Mutations of the alpha2 subunit are a major cause of congenital muscular dystrophy. To determine whether the costameres are altered as a result of laminin alpha2-mutations, the skeletal muscle surface of a dystrophic mouse (dy(2J)/dy(2J)) lacking the alpha2-LN domain was examined by confocal and widefield deconvolution immunomicroscopy. Although the dy(2J) dystrophic fibers possessed a normal-appearing distribution of alpha2-laminins and alpha-dystroglycan within a rectilinear costameric lattice at 6.5 weeks of age, by 11 weeks the surface architecture of these components were found to be disorganized, with frequent effacement of the circumferential and longitudinal lattice striations. The defect in the lattice organization was also noted to be a characteristic of type IV collagen, nidogen, perlecan, beta1(D)-integrin, dystrophin and vinculin. The development of this pattern change occurring only after birth suggests that although alpha2-laminins are not essential for the initial assembly of the costameric framework, they play a role in maintaining the stability and organization of the framework.

Basement Membrane - cytology

Dystroglycans - metabolism

Receptors, Cell Surface - metabolism

Muscle, Skeletal - metabolism

Muscle, Skeletal - cytology

Basement Membrane - metabolism

Basement Membrane - pathology

Muscular Dystrophy, Animal - pathology

Laminin - deficiency

Animals

Aging - physiology

Sarcolemma - pathology

Mice, Mutant Strains

Muscular Dystrophy, Animal - metabolism

Cytoskeleton - metabolism

Dystroglycans - chemistry

Dystroglycans - genetics

Mice

Laminin - metabolism

Muscle, Skeletal - pathology

Sarcolemma - metabolism

Microscopy, Fluorescence

Sequence Deletion - genetics

Details

Title: Subtitle: Loss of basement membrane, receptor and cytoskeletal lattices in a laminin-deficient muscular dystrophy
Creators: Peter D Yurchenco - Department of Pathology & Laboratory Medicine, UMDNJ-Robert Wood Johnson Medical School, Piscataway, NJ 08854, USA. yurchenc@umdnj.edu
Yi-Shan Cheng
Kevin Campbell
Shaohua Li
Resource Type: Journal article
Publication Details: Journal of cell science, Vol.117(Pt 5), pp.735-742
DOI: 10.1242/jcs.00911
PMID: 14734655
NLM abbreviation: J Cell Sci
ISSN: 0021-9533
eISSN: 1477-9137
Publisher: England
Grant note: NS38469 / NINDS NIH HHS R37 DK036425 / NIDDK NIH HHS
Language: English
Date published: 02/15/2004
Academic Unit: Neurology; Molecular Physiology and Biophysics; Iowa Neuroscience Institute
Record Identifier: 9984020742802771

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