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(American Journal of Pathology. 2001;159:1581-1592.)
© 2001 American Society for Investigative Pathology

Animal Model

Lack of Cytosolic and Transmembrane Domains of Type XIII Collagen Results in Progressive Myopathy

Ari-Pekka Kvist^*, Anne Latvanlehto^*, Malin Sund^*, Lauri Eklund^*, Timo Väisänen^*, Pasi Hägg^*, Raija Sormunen, Jyrki Komulainen, Reinhard Fässler and Taina Pihlajaniemi^*

From the Department of Medical Biochemistry,^*
Collagen Research Unit, and the Department of Pathology,
Biocenter Oulu, University of Oulu, Oulu, Finland; the LIKES Research Center for Sport and Health Sciences,
University Campus, Jyväskylä, Finland; and the Department of Experimental Pathology,
Lund University, Lund, Sweden

Type XIII collagen is a type II transmembrane protein found at many sites of cell adhesion in tissues. Homologous recombination was used to generate a transgenic mouse line (Col13a1^N/N) that expresses N-terminally altered type XIII collagen molecules lacking the short cytosolic and transmembrane domains but retaining the large collagenous ectodomain. The mutant molecules were correctly transported to focal adhesions in cultured fibroblasts derived from the Col13a1^N/N mice, but the cells showed decreased adhesion when plated on type IV collagen. These mice were viable and fertile, and in immunofluorescence stainings the mutant protein was located in adhesive tissue structures in the same manner as normal 1(XIII) chains. In immunoelectron microscopy of wild-type mice type XIII collagen was detected at the plasma membrane of skeletal muscle cells whereas in the mutant mice the protein was located in the adjacent extracellular matrix. Affected skeletal muscles showed abnormal myofibers with a fuzzy plasma membrane-basement membrane interphase along the muscle fiber and at the myotendinous junctions, disorganized myofilaments, and streaming of z-disks. The findings were progressive and the phenotype was aggravated by exercise. Thus type XIII collagen seems to participate in the linkage between muscle fiber and basement membrane, a function impaired by lack of the cytosolic and transmembrane domains.

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