title: Intrinsic laryngeal muscles are spared from degeneration in the dy3K/dy3K mouse model of congenital muscular dystrophy type 1A.
abstract: Deficiency of laminin alpha2 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 alpha2 chain absence. No muscle degeneration was evident; expression of various laminin chains was similar to that of limb muscles, and sustained integrin alpha7B expression was noted in laminin alpha2 chain-deficient ILM. We conclude that ILM are spared in MDC1A.
authors.affiliation: Department of Experimental Medical Science, Lund University, Muscle Biology Unit, 221 84 Lund, Sweden.
authors.forename: Mattias
authors.initials: M
authors.lastname: Häger
authors.forename: Madeleine
authors.initials: M
authors.lastname: Durbeej
mesh_terms: D000818:Animals; D001485:Basement Membrane / Q000378:metabolism / Q000648:ultrastructure; D001665:Binding Sites / Q000235:genetics; D019070:Cell Lineage / Q000235:genetics; D019610:Cytoprotection / Q000235:genetics; D004195:Disease Models, Animal; D016189:Dystrophin / Q000235:genetics; D020022:Genetic Predisposition to Disease / Q000235:genetics; D016023:Integrins / Q000235:genetics / Q000378:metabolism; D007797:Laminin / Q000235:genetics*; D007821:Laryngeal Muscles / Q000196:embryology / Q000378:metabolism / Q000473:pathology*; D051379:Mice; D018345:Mice, Knockout; D009137:Muscular Dystrophy, Animal / Q000151:congenital* / Q000235:genetics / Q000473:pathology*; D011485:Protein Binding / Q000235:genetics
chemical_list: D016189:Dystrophin; D016023:Integrins; D007797:Laminin; C102645:laminin alpha 2; C082908:integrin alpha7beta1