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Intrinsic laryngeal muscles are spared from degeneration in the dy 3k / dy 3k mouse model of congenital muscular dystrophy type 1A
Author(s) -
Häger Mattias,
Durbeej Madeleine
Publication year - 2009
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.21209
Subject(s) - laminin , congenital muscular dystrophy , muscular dystrophy , anatomy , biology , medicine , extracellular matrix , genetics
Deficiency of laminin α2 chain leads to a severe form of congenital muscular dystrophy (MDC1A). Here, we analyzed whether the intrinsic laryngeal muscles (ILM) are spared in the dy 3K / dy 3K mouse model of complete laminin α2 chain absence. No muscle degeneration was evident; expression of various laminin chains was similar to that of limb muscles, and sustained integrin α7B expression was noted in laminin α2 chain‐deficient ILM. We conclude that ILM are spared in MDC1A. Muscle Nerve 39: 91–94, 2009