Leopard syndrome associated with hyperelastic skin: Analysis of collagen metabolism in cultured skin fibroblasts

Authors
Ohkura, T Ohnishi, Y Kawada, A Tajima, S Ishibashi, A Ono, K
Citation
T. Ohkura et al., Leopard syndrome associated with hyperelastic skin: Analysis of collagen metabolism in cultured skin fibroblasts, DERMATOLOGY, 198(4), 1999, pp. 385-387
Citations number
15
Categorie Soggetti
Dermatology
Journal title
DERMATOLOGY
ISSN journal
10188665 → ACNP
Volume
198
Issue
4
Year of publication
1999
Pages
385 - 387
Database
ISI
SICI code
1018-8665(1999)198:4<385:LSAWHS>2.0.ZU;2-I
Abstract
We present a patient with Leopard syndrome and hyperelastic skin. Biochemic al analysis using cultured skin fibroblasts showed normal type III and V co llagen synthesis, lysyl hydroxylation level of type I procollagen and proce ssing of pro-alpha(1) and alpha(2)(I). Our results suggest that molecular d efects of hyperelasticity in Leopard syndrome are not related to abnormal c ollagen metabolism, although not all steps of collagen synthesis have been investigated.