Distinct roles for laminin globular domains in laminin alpha1 chain mediated rescue of murine laminin alpha2 chain deficiency.

PloS One
Kinga I GawlikMadeleine Durbeej

Abstract

Laminin alpha2 chain mutations cause congenital muscular dystrophy with dysmyelination neuropathy (MDC1A). Previously, we demonstrated that laminin alpha1 chain ameliorates the disease in mice. Dystroglycan and integrins are major laminin receptors. Unlike laminin alpha2 chain, alpha1 chain binds the receptors by separate domains; laminin globular (LG) domains 4 and LG1-3, respectively. Thus, the laminin alpha1 chain is an excellent tool to distinguish between the roles of dystroglycan and integrins in the neuromuscular system. Here, we provide insights into the functions of laminin alpha1LG domains and the division of their roles in MDC1A pathogenesis and rescue. Overexpression of laminin alpha1 chain that lacks the dystroglycan binding LG4-5 domains in alpha2 chain deficient mice resulted in prolonged lifespan and improved health. Importantly, diaphragm and heart muscles were corrected, whereas limb muscles were dystrophic, indicating that different muscles have different requirements for LG4-5 domains. Furthermore, the regenerative capacity of the skeletal muscle did not depend on laminin alpha1LG4-5. However, this domain was crucial for preventing apoptosis in limb muscles, essential for myelination in peripheral nerve and ...Continue Reading

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Citations

Mar 23, 2011·Cold Spring Harbor Perspectives in Biology·Peter D Yurchenco
Oct 19, 2011·Journal of Biomedicine & Biotechnology·Jessica D Gumerson, Daniel E Michele
Jul 30, 2011·Skeletal Muscle·Kinga I Gawlik, Madeleine Durbeej
May 24, 2011·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Sijo MathewRoy Zent
Nov 19, 2010·Human Molecular Genetics·Virginie CarmignacMadeleine Durbeej
Mar 19, 2014·The American Journal of Pathology·Zandra KörnerMadeleine Durbeej
Jan 17, 2012·Muscle & Nerve·Ranjan GuptaTahseen Mozaffar
Aug 13, 2011·Developmental Neurobiology·Holly Colognato, Iva D Tzvetanova
Jul 10, 2012·Neuromuscular Disorders : NMD·Chris J Moore, Steve J Winder
Nov 17, 2012·Cell Adhesion & Migration·Johan Holmberg, Madeleine Durbeej
Oct 4, 2016·Cellular and Molecular Life Sciences : CMLS·Yao Yao
Feb 9, 2017·Stem Cell Research & Therapy·Jyoti GautamYao Yao
May 28, 2020·Frontiers in Molecular Neuroscience·Kinga I Gawlik, Madeleine Durbeej
Apr 16, 2019·Frontiers in Molecular Biosciences·Christopher E DempseyAndrea Brancaccio
Apr 16, 2021·Metabolism: Clinical and Experimental·Anna GoddiRonald N Cohen
Jul 7, 2012·Journal of Cell Science·Karen K McKeePeter D Yurchenco

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Methods Mentioned

BETA
glycosylation
transgenic
PCR
fluorescence microscopy
transmission electron microscopy

Software Mentioned

Adobe Photoshop
Openlab

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