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Yurchenco PD, McKee KK. Linker Protein Repair of LAMA2 Dystrophic Neuromuscular Basement Membranes. Front Mol Neurosci. 2019;12:305doi: 10.3389/fnmol.2019.00305.
Yurchenco, P. D., & McKee, K. K. (2019). Linker Protein Repair of LAMA2 Dystrophic Neuromuscular Basement Membranes. Frontiers in molecular neuroscience, 12305. https://doi.org/10.3389/fnmol.2019.00305
Yurchenco, Peter D, and McKee, Karen K. "Linker Protein Repair of LAMA2 Dystrophic Neuromuscular Basement Membranes." Frontiers in molecular neuroscience vol. 12 (2019): 305. doi: https://doi.org/10.3389/fnmol.2019.00305
Yurchenco PD, McKee KK. Linker Protein Repair of LAMA2 Dystrophic Neuromuscular Basement Membranes. Front Mol Neurosci. 2019 Dec 13;12:305. doi: 10.3389/fnmol.2019.00305. eCollection 2019. PMID: 31920536; PMCID: PMC6923227.
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Front Mol Neurosci. 2019 Dec 13;12:305. doi: 10.3389/fnmol.2019.00305. eCollection 2019.

Linker Protein Repair of LAMA2 Dystrophic Neuromuscular Basement Membranes.

Frontiers in molecular neuroscience

Peter D Yurchenco, Karen K McKee

Affiliations

  1. Department of Pathology and Laboratory Medicine, Rutgers Robert Wood Johnson Medical School, Rutgers University, Piscataway, NJ, United States.

PMID: 31920536 PMCID: PMC6923227 DOI: 10.3389/fnmol.2019.00305

Abstract

An understanding of basement membrane (BM) assembly at a molecular level provides a foundation with which to develop repair strategies for diseases with defects of BM structure. As currently understood, laminins become anchored to cell surfaces through receptor-mediated interactions and polymerize. This provisional matrix binds to proteoglycans, nidogens and type IV collagen to form a mature BM. Identification of BM binding domains and their binding targets has enabled investigators to engineer proteins that link BM components to modify and improve their functions. This approach is illustrated by the development of two linker proteins to repair the LAMA2-deficient muscular dystrophy (LAMA2-MD). Dystrophy-causing mutations of the

Copyright © 2019 Yurchenco and McKee.

Keywords: adeno-associated virus; extracellular matrices; gene-therapy; muscle; peripheral nerve; self-assembly

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