Characterization of Adiposity and Metabolism in Lmna-Deficient Mice

Dunnigan's Familial Partial Lipodystrophy (FPLD) is an autosomal dominant disease characterized by regional fat loss and insulin resistance. FPLD is caused by mutations in the LMNA gene, which encodes intermediate filaments of the nuclear lamina. Different LMNA mutations cause Emery–Dreifuss mu...

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Bibliographic Details
Published in:Biochemical and biophysical research communications 2002-03, Vol.291 (3), p.522-527
Main Authors: Cutler, Dedra A., Sullivan, Teresa, Marcus-Samuels, Bernice, Stewart, Colin L., Reitman, Marc L.
Format: Article
Language:English
Subjects:
adipose tissue
Adipose Tissue - metabolism
animal models
Animals
Blood Glucose - analysis
diabetes
Disease Models, Animal
Dunnigan's familial partial lipodystrophy
Eating
Female
Insulin - blood
lamin
Lamins
lipodystrophy
Lipodystrophy - blood
Lipodystrophy - etiology
Lipodystrophy - metabolism
Lmna gene
Male
Mice - genetics
Mice, Knockout
Muscle, Skeletal - metabolism
Muscular Dystrophies - etiology
Muscular Dystrophies - metabolism
Nuclear Proteins - genetics
Nuclear Proteins - physiology
Phenotype
RNA, Messenger - biosynthesis
Sex Factors
Tissue Distribution
Triglycerides - blood
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Summary:Dunnigan's Familial Partial Lipodystrophy (FPLD) is an autosomal dominant disease characterized by regional fat loss and insulin resistance. FPLD is caused by mutations in the LMNA gene, which encodes intermediate filaments of the nuclear lamina. Different LMNA mutations cause Emery–Dreifuss muscular dystrophy and/or a dilated cardiomyopathy. It is not known how LMNA mutations cause any of the disease phenotypes. Here we measure physical and metabolic characteristics of Lmna−/− and +/− mice to determine their usefulness as models for FPLD. Lmna−/− mice, which die prematurely of muscular dystrophy, have little fat, but do not show the insulin resistance characteristic of FPLD. Lmna+/− mice, despite treatment with a high fat diet, do not have decreased fat stores or metabolic features of FPLD. We also show, in mice, that Lmna transcripts are expressed at high levels in muscle and adipose tissue, but do not vary by body region or sex. In conclusion, Lmna+/− and −/− mice do not mimic Dunnigan's FPLD, and differential expression of lamins A and C does not appear to contribute to sex- or tissue-specific LMNA phenotypes.
ISSN:0006-291X
1090-2104
DOI:10.1006/bbrc.2002.6466