Novel collagen VI alpha chains distribution in murine skeletal muscle: possible implications for neuromuscular disorders (Abstract/Poster in atti di convegno)

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  • Novel collagen VI alpha chains distribution in murine skeletal muscle: possible implications for neuromuscular disorders (Abstract/Poster in atti di convegno) (literal)
Anno
  • 2008-01-01T00:00:00+01:00 (literal)
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  • Sabatelli P, Wagener R, Paulsson M, Gara SK, Squarzoni S, Zamparelli A, Grumati P, Urciuolo A, Merlini L, Ferlini A, Bonaldo P (2008)
    Novel collagen VI alpha chains distribution in murine skeletal muscle: possible implications for neuromuscular disorders
    in XXXIX Congresso della Società Italiana di Neurologia, 18 - 22 Ottobre 2008, Centro Congressi - Mostra d’Oltremare – Napoli, Napoli
    (literal)
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  • Sabatelli P, Wagener R, Paulsson M, Gara SK, Squarzoni S, Zamparelli A, Grumati P, Urciuolo A, Merlini L, Ferlini A, Bonaldo P (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#descrizioneSinteticaDelProdotto
  • Collagen type VI is a major extracellular matrix (ECM) protein expressed in several tissues, including skeletal muscle and skin. Until few months ago, it was thought to consist of three genetically distinct alpha chains (±1, ±2, ±3), secreted in the ECM where they form an extended microfilamentous network, associated with the basal lamina of muscle fibers. Mutations in COL6A1, COL6A2 and COL6A3 genes cause Ullrich congenital muscular dystrophy (UCMD) and Bethlem myopathy. Recent studies identified three new chains, ±4, ±5 and ±6, which structurally resemble the collagen VI a3 chain, encoded by three different genes, COL6A4, COL6A5 and COL6A6, respectively. In mouse tissues, ±6 and ±5 chains are expressed in skeletal muscle, while ±4 is restricted to colon. To gain insight into the function of novel ±5 and ±6 chains in muscle, we performed an immunoelectron microscopy study using specific antibodies against ±5 or ±6 chains and compared the distribution with the ±1-2-3 known chains, in murine skeletal muscle. In quadriceps muscle, ±1-2-3 chains were localized at the basement membrane of muscle fibers and capillary vessels, and in intramuscular peripheral nerves. The ±6 chain was restricted to the reticular lamina of the basement membrane of muscle fibers: colloidal gold particles were mainly associated with the microfibrillar network that encircles collagen fibers enriching the lamina densa. In contrast with anti ±1-2-3 chains antibody, alpha6 chain was absent at the interface of reticular lamina and the lamina densa and in the basement membrane of blood capillary wall and peripheral nerves. Alpha 5 chain showed an uneven distribution in the interstitium while was localized at the perineurium of intramuscular peripheral nerves. Interestingly, a strong labelling was also detected at the basement membrane of neuromuscular junctions. Our data indicate a differential distribution of novel ±6 and ±5 chains in skeletal muscle with respect to the widely distributed ±1-2-3 classical chains, suggesting possible specific roles of new alpha chains in very specialised areas. Considering that, collagen VI ±6 chain is expressed in skeletal muscle, and that in a growing number of BM/UCMD patients COL6A1, COL6A2 and COL6A3 mutations has been excluded, COL6A6 may represent a new candidate gene for congenital muscular dystrophy. The selective expression of ±5 chain at the neuromuscular junctions and perineurium adds new possible perspectives in neurological diseases. (literal)
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  • IGM-CNR Unit of Bologna (literal)
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  • Novel collagen VI alpha chains distribution in murine skeletal muscle: possible implications for neuromuscular disorders (literal)
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