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Congenital Lipodystrophies and Dyslipidemias

  • Rare Diseases and Lipid Metabolism (JAG López, Section Editor)
  • Published:
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Abstract

Lipodystrophies are rare acquired and genetic disorders characterized by the selective loss of adipose tissue. One key metabolic feature of patients with congenital inherited lipodystrophy is hypertriglyceridemia. The precise mechanisms by which the lack of adipose tissue causes dyslipidemia remain largely unknown. In recent years, new insights have arisen from data obtained in vitro in adipocytes, yeast, drosophila, and very recently in several genetically modified mouse models of generalized lipodystrophy. A common metabolic pathway involving accelerated lipolysis and defective energy storage seems to contribute to the dyslipidemia associated with congenital generalized lipodystrophy syndromes, although the pathophysiological changes may vary with the nature of the mutation involved. Therapeutic management of dyslipidemia in patients with lipodystrophy is primarily based on specific approaches using recombinant leptin therapy. Preclinical studies suggest a potential efficacy of thiazolidinediones that remains to be assessed in dedicated clinical trials.

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Acknowledgments

This work was supported by grants from the Institut National de la Santé et de la Recherche Médicale (Inserm), the French Ministère de l’Enseignement Supérieur et de la Recherche, and French associations (Aide aux Jeunes Diabétiques, Fondation de France, Fondation GenaVie, Association de Langue Française pour l’Etude du Diabète et des Maladies Métaboliques/Société Francophone du Diabète, and Assocation pour la Recherche Diabète).

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Conflict of Interest

Xavier Prieur, Cedric Le May, Jocelyne Magré, and Bertrand Cariou declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

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Authors and Affiliations

  1. INSERM U1087-CNRS UMR 6291, L’institut du Thorax, 8 quai Moncousu, 44007, Nantes Cedex 1, France

    Xavier Prieur, Cedric Le May, Jocelyne Magré & Bertrand Cariou

  2. Université de Nantes, UFR des sciences et techniques, 2 Chemin de la Houssinière, 44300, Nantes, France

    Xavier Prieur

  3. Université de Nantes, Faculté de Médecine, 1 Rue Gaston Veïl, 44000, Nantes, France

    Bertrand Cariou

  4. Department of endocrinology, Nantes University Hospital, HGRL Saint-Herblain, 44093, Cedex 1, France

    Bertrand Cariou

  5. IRT-UN, L’institut du Thorax, Unité INSERM UMR 1087-CNRS 6291, 8 quai Moncousu, 44007, Nantes Cedex 1, France

    Xavier Prieur

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  1. Xavier Prieur
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  2. Cedric Le May
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  3. Jocelyne Magré
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  4. Bertrand Cariou
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Correspondence to Xavier Prieur.

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This article is part of the Topical Collection on Rare Diseases and Lipid Metabolism

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Prieur, X., Le May, C., Magré, J. et al. Congenital Lipodystrophies and Dyslipidemias. Curr Atheroscler Rep 16, 437 (2014). https://doi.org/10.1007/s11883-014-0437-x

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