A. Malandrini et al., LOCALIZATION OF THE LAMININ ALPHA-2 CHAIN IN NORMAL HUMAN SKELETAL-MUSCLE AND PERIPHERAL-NERVE - AN ULTRASTRUCTURAL IMMUNOLABELING STUDY, Acta Neuropathologica, 93(2), 1997, pp. 166-172
A particular form of congenital muscular dystrophy is associated with
a deficiency of the tissue-specific basement membrane protein laminin
alpha 2. A more precise knowledge of the normal distribution and local
ization of laminin alpha 2 would be useful in further elucidating the
development of this disorder. In this study we used specific electron
microscopic techniques, i.e., thin-section fracture labeling and cryou
ltramicrotomy in combination with immunogold labeling for laminin alph
a 2, to determine its ultrastructural localization in normal human mus
cle and peripheral nerve. Both in muscle and in peripheral nerve, lami
nin alpha 2 is found to be associated solely with the basal lamina of
myofibers and Schwann cells, respectively. Of special interest is the
finding that in peripheral nerve, laminin alpha 2 is associated only w
ith myelinated and not with unmyelinated nerve fibers.