Increased chloride efflux in fibroblasts from X‐linked muscular dystrophies and clones from Duchenne carriers

FEBS Letters

Lenaz, Giorgio¹  Rugolo, Michela¹  Rocchi, Mariano²  Romeo, Giovanni² 

[1] Dipartimento di Biologia, Istituto Botanico, Via Irnerio 42, 40126 BolognaItaly;Laboratorio di Genetica Molecolare, Istituto G. Gaslini, Via 5 Maggio 39, 16148 Genova, Italy

关键词: Cl− efflux;    Fibroblast;    X-linked muscular dystrophy;    Duchenne muscular dystrophy;    Becker muscular dystrophy;    Emery-Dreifuss muscular dystrophy;    (Human);    DMD;    Duchenne muscular dystrophy;    BMD;    Becker muscular dystrophy;    EDMD;    Emery-Dreifuss muscular dystrophy;   

DOI  :  10.1016/0014-5793(87)81367-4

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

Previous studies have suggested an increased chloride membrane permeability in Duchenne muscular dystrophy (DMD) fibroblasts. We report that an increased chloride efflux with respect to controls is present not only in fibroblasts from DMD, but also from two other X-linked muscular dystrophies, Becker and Emery-Dreifuss, as well as in clones from DMD carrier females. The latter observation suggests that, at least in DMD, the increased chloride efflux phenotype might be subject to lyonization.

【 授权许可】

Unknown