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FIBROBLAST CULTURES IN DUCHENNE MUSCULAR DYSTROPHY
Author(s) -
Ionasescu Victor,
LaraBraud Carolyn,
Zellweger Hans,
Ionasescu Rebeca,
Burmeister Leon
Publication year - 1977
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.1977.tb05659.x
Subject(s) - fibroblast , collagenase , duchenne muscular dystrophy , collagen vi , extracellular , polyacrylamide gel electrophoresis , proline , muscular dystrophy , microbiology and biotechnology , microbial collagenase , biology , chemistry , cell culture , biochemistry , medicine , extracellular matrix , genetics , enzyme , amino acid
Primary skin fibroblast cultures were grown from forearm pinch skin biopsies obtained from 24 patients with Duchenne muscular dystrophy (DMD) and ten normal controls matched for sex and age. The first subcultures were grown for 7 days and incubated with L‐( 3 H)‐proline for 24 hours. Intracellular collagen incorporation was significantly decreased (2.2 ×) and extracellular collagen incorporation significantly increased (1.8 ×) in fibroblast cultures from patients with DMD by both collagenase assay and polyacrylamide gel electrophoresis. The synthesis of noncollagen proteins showed low values from the DMD fibroblast cultures. The alterations in synthesis and secretion of collagen and noncollagen proteins were characteristic only for the log phase of DMD fibroblasts.