Article
Extracellular matrix remodeling and transforming growth factor-β signaling abnormalities induced by lamin A/C variants that cause lipodystrophy.
Journal of lipid research - 1 Jan 2017
Le Dour Caroline, Wu Wei, Béréziat Véronique, Capeau Jacqueline, Vigouroux Corinne, Worman Howard J
Abstract excerpt
Mutations in the lamin A/C gene encoding nuclear lamins A and C (lamin A/C) cause familial partial lipodystrophy type 2 (FPLD2) and related lipodystrophy syndromes. These are mainly characterized by redistribution of adipose tissue associated with insulin resistance. Several reports suggest that alterations in the extracellular matrix of adipose tissue leading to fibrosis play a role in the pathophysiology of...
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