title: Proline incorporation by cultured skin fibroblasts from patients with duchenne muscular dystrophy.
abstract: The incorporation of proline by cultured skin fibroblasts derived from normal individuals and patients with Duchenne muscular dystrophy (DMD) was analyzed by SDS-gel electrophoresis combined with a double-labeling procedure [Pediatr. Res., 12: 887 (1978)]. DMD fibroblasts showed increased proline incorporation into protein of approximately 130 000 daltons which could be degraded partially by collagenase. This difference was observed only at 7 days after seeding, and may be due to slight differences in growth rate as comparison of cells harvested at 2 and 7 days indicated that increased proline incorporation into high molecular weight protein was associated with day 7 cells, whether normal or DMD. During 14 days in culture normal and DMD strains did not differ in protein content, doubling time or incorporation of [3H]leucine and [14C]proline into cellular protein, although the ratio of [14C]proline to [3H]leucine incorporated was greater for DMD fibroblasts at 7 days. Incorporation of [14C]proline and [3H]leucine into extracellular proteins was greater in DMD fibroblast cultures. These subtle differences support the hypothesis that the DMD gene is expressed in fibroblasts.
mesh_terms: D002454:Cell Differentiation; D003470:Culture Media; D046508:Culture Techniques; D004591:Electrophoresis, Polyacrylamide Gel; D005347:Fibroblasts / Q000166:cytology / Q000378:metabolism; D006801:Humans; D007930:Leucine / Q000378:metabolism; D008970:Molecular Weight; D009136:Muscular Dystrophies / Q000378:metabolism*; D011392:Proline / Q000378:metabolism*; D011506:Proteins / Q000378:metabolism; D012867:Skin / Q000166:cytology / Q000378:metabolism*
chemical_list: D003470:Culture Media; D011506:Proteins; D011392:Proline; D007930:Leucine