{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T02:17:16Z","timestamp":1791166636425,"version":"4.1.0"},"reference-count":82,"publisher":"The Company of Biologists","issue":"11-12","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2009,11,5]]},"abstract":"<jats:p>Insulin resistance is a major factor in the pathogenesis of type 2 diabetes and underpins the strong association between obesity and diabetes. Paradoxically, the metabolic consequences of having \u2018too much\u2019 fat (obesity) are remarkably similar to those of having \u2018too little\u2019 fat (lipodystrophy): a finding that has generated considerable interest in a rare disease. In both cases, excess energy accumulates as lipid in ectopic sites such as the liver (fatty liver) and skeletal muscle, where it plays a central role in the pathogenesis of insulin resistance, dyslipidemia and type 2 diabetes. Human lipodystrophies are characterised by a total or partial deficiency of body fat, and may be inherited or acquired in origin. Genetically engineered mice with generalised lipodystrophy manifest many of the features of the human disorder, including hyperphagia, fatty liver, hypertriglyceridaemia, insulin resistance and type 2 diabetes, providing a useful tractable model of the human disorder. Partial lipodystrophy, which causes similar, albeit milder, metabolic problems in humans has been more difficult to mimic in the mouse. This review discusses key translational studies in mice with generalised lipodystrophy, including fat transplantation and the use of recombinant leptin replacement therapy. These studies have been instrumental in advancing our understanding of the underlying molecular pathogenesis of ectopic lipid accumulation and insulin resistance, and have prompted the initiation and subsequent adoption of leptin replacement therapy in human lipodystrophies. This review also considers the possible reasons for the apparent difficulties in generating mouse models of partial lipodystrophy, such as interspecies differences in the distribution of fat depots and the apparent lack of sexual dimorphism in fat mass and distribution in mice compared with the dramatic differences present in adult humans.<\/jats:p>","DOI":"10.1242\/dmm.002907","type":"journal-article","created":{"date-parts":[[2009,11,5]],"date-time":"2009-11-05T21:31:57Z","timestamp":1257456717000},"page":"554-562","source":"Crossref","is-referenced-by-count":96,"title":["Mouse models of inherited lipodystrophy"],"prefix":"10.1242","volume":"2","author":[{"given":"David B.","family":"Savage","sequence":"first","affiliation":[{"name":"Metabolic Research Laboratories, Institute of Metabolic Science, University of Cambridge, Addenbrooke\u2019s Hospital, Hills Road, Cambridge CB2 8RP, 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fasting","volume":"382","author":"Ahima","year":"1996","journal-title":"Nature"},{"key":"2021042500081072400_b4-0020554","doi-asserted-by":"crossref","first-page":"414","DOI":"10.1172\/JCI200419511","article-title":"Site and mechanism of leptin action in a rodent form of congenital lipodystrophy","volume":"113","author":"Asilmaz","year":"2004","journal-title":"J Clin Invest"},{"key":"2021042500081072400_b5-0020554","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.ddmod.2007.10.003","article-title":"Mouse models of lipodystrophy key reagents for the understanding of the metabolic syndrome","volume":"4","author":"Asterholm","year":"2007","journal-title":"Drug Discov Today Dis Models"},{"key":"2021042500081072400_b6-0020554","doi-asserted-by":"crossref","first-page":"880","DOI":"10.1038\/47254","article-title":"Dominant negative mutations in human PPARgamma associated with severe insulin resistance, diabetes mellitus and hypertension","volume":"402","author":"Barroso","year":"1999","journal-title":"Nature"},{"key":"2021042500081072400_b7-0020554","doi-asserted-by":"crossref","first-page":"2297","DOI":"10.1056\/NEJMoa060326","article-title":"A placebo-controlled trial of pioglitazone in subjects with nonalcoholic steatohepatitis","volume":"355","author":"Belfort","year":"2006","journal-title":"N Engl J Med"},{"key":"2021042500081072400_b8-0020554","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1016\/S1043-2760(01)00524-0","article-title":"ACRP30\/adiponectin: an adipokine regulating glucose and lipid metabolism","volume":"13","author":"Berg","year":"2002","journal-title":"Trends Endocrinol Metab"},{"key":"2021042500081072400_b9-0020554","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1016\/j.cmet.2007.11.013","article-title":"Hepatic insulin resistance is sufficient to produce dyslipidemia and susceptibility to atherosclerosis","volume":"7","author":"Biddinger","year":"2008","journal-title":"Cell Metab"},{"key":"2021042500081072400_b10-0020554","doi-asserted-by":"crossref","first-page":"796","DOI":"10.1016\/j.biochi.2009.01.011","article-title":"Seipin deficiency alters fatty acid Delta9 desaturation and lipid droplet formation in Berardinelli-Seip congenital lipodystrophy","volume":"91","author":"Boutet","year":"2009","journal-title":"Biochimie"},{"key":"2021042500081072400_b11-0020554","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1016\/j.cmet.2007.12.009","article-title":"Selective versus total insulin resistance: a pathogenic paradox","volume":"7","author":"Brown","year":"2008","journal-title":"Cell Metab"},{"key":"2021042500081072400_b12-0020554","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1093\/hmg\/9.1.109","article-title":"Nuclear lamin A\/C R482Q mutation in canadian kindreds with Dunnigan-type familial partial 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Med"},{"key":"2021042500081072400_b47-0020554","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1038\/nm1262","article-title":"Fat apoptosis through targeted activation of caspase 8, a new mouse model of inducible and reversible lipoatrophy","volume":"11","author":"Pajvani","year":"2005","journal-title":"Nat Med"},{"key":"2021042500081072400_b48-0020554","first-page":"503","article-title":"Leptin levels, beta-cell function, and insulin sensitivity in families with congenital and acquired generalized lipoatropic diabetes","volume":"83","author":"Pardini","year":"1998","journal-title":"J Clin Endocrinol Metab"},{"key":"2021042500081072400_b49-0020554","doi-asserted-by":"crossref","first-page":"2055","DOI":"10.2337\/db08-0184","article-title":"The human lipodystrophy gene BSCL2\/seipin may be essential for normal adipocyte 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J. Obes. Relat. Metab. Disord"},{"key":"2021042500081072400_b57-0020554","doi-asserted-by":"crossref","first-page":"436","DOI":"10.1097\/01.mco.0000232904.82038.db","article-title":"Metabolic consequences of lipodystrophy in mouse models","volume":"9","author":"Reue","year":"2006","journal-title":"Curr Opin Clin Nutr Metab Care"},{"key":"2021042500081072400_b58-0020554","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1016\/j.cell.2008.09.036","article-title":"Identification of white adipocyte progenitor cells in vivo","volume":"135","author":"Rodeheffer","year":"2008","journal-title":"Cell"},{"key":"2021042500081072400_b59-0020554","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1016\/S1097-2765(00)80211-7","article-title":"PPAR gamma is required for the differentiation of adipose tissue in vivo and in vitro","volume":"4","author":"Rosen","year":"1999","journal-title":"Mol 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Rev"},{"key":"2021042500081072400_b63-0020554","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1111\/j.1651-2227.1996.tb14262.x","article-title":"Generalized lipodystrophy, congenital and acquired (lipoatrophy)","volume":"413","author":"Seip","year":"1996","journal-title":"Acta Paediatr Suppl"},{"key":"2021042500081072400_b64-0020554","doi-asserted-by":"crossref","first-page":"581","DOI":"10.1172\/JCI28003","article-title":"PPAR gamma and human metabolic disease","volume":"116","author":"Semple","year":"2006","journal-title":"J Clin Invest"},{"key":"2021042500081072400_b65-0020554","first-page":"315","article-title":"Postreceptor insulin resistance contributes to human dyslipidemia and hepatic steatosis","volume":"119","author":"Semple","year":"2009","journal-title":"J Clin Invest"},{"key":"2021042500081072400_b66-0020554","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1038\/72807","article-title":"LMNA, encoding lamin A\/C, is mutated in partial 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livers of lipodystrophic and ob\/ob mice","volume":"6","author":"Shimomura","year":"2000","journal-title":"Mol Cell"},{"key":"2021042500081072400_b70-0020554","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1172\/JCI10583","article-title":"Cellular mechanisms of insulin resistance","volume":"106","author":"Shulman","year":"2000","journal-title":"J Clin Invest"},{"key":"2021042500081072400_b71-0020554","doi-asserted-by":"crossref","first-page":"5433","DOI":"10.1210\/jc.2003-030835","article-title":"Phenotypic heterogeneity in body fat distribution in patients with congenital generalized lipodystrophy caused by mutations in the AGPAT2 or seipin genes","volume":"88","author":"Simha","year":"2003","journal-title":"J Clin Endocrinol Metab"},{"key":"2021042500081072400_b72-0020554","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1016\/S0092-8674(01)00240-9","article-title":"Obesity and the regulation of energy 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