Skeletal muscle morphology and exercise response in congenital generalized lipodystrophy.

• DOI: 10.2337/diacare.23.10.1545
• Publication Date: 2007-03-05T22:51:27Z
• Authors: A Garg; J Stray-Gundersen; D Parsons; L A Bertocci
• Keywords: Lipodystrophy; Morphology

OBJECTIVE: Congenital generalized lipodystrophy (CGL) is an autosomal recessive genetic disorder characterized by almost complete absence of adipose tissue, muscular appearance, and severe insulin resistance since birth. We investigated whether in CGL patients associated with abnormal muscle morphology increased muscularity imparts strength exercise capacity RESEARCH DESIGN AND METHODS: obtained quadriceps biopsies to study fiber types capillary density three African-American women (aged 17-20 years) CGL. also assessed strength, metabolism, maximal O2 consumption the patients. RESULTS: Quadriceps revealed a markedly higher percentage type II (fast-twitch glycolytic) fibers versus sedentary young (75-78 vs. 47-57%, respectively). The capillary-to-fiber ratio (2.7-3.0), however, was normal. Cross-sectional areas I (slow-twitch oxidative) (1,262-2,685 microm2) (2,304-3,594 were far below normal values (3,811-4,310 3,115-4,193 microm2, respectively), suggesting hyperplasia but not hypertrophy as measured Cybex, average; (23-32 ml x kg(-1) min(-1)) average. 31P nuclear magnetic resonance spectroscopy forearm muscles pH metabolic responses static dynamic exercises. CONCLUSIONS: conclude that proportion reduced density. Increased due strength.