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首页> 外文期刊>Proceedings of the National Academy of Sciences of the United States of America >Transient Ciliogenesis Involving Bardet-biedl Syndrome Proteins Is A Fundamental Characteristic Of Adipogenic Differentiation
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Transient Ciliogenesis Involving Bardet-biedl Syndrome Proteins Is A Fundamental Characteristic Of Adipogenic Differentiation

机译：涉及Bardet-Biedl综合征蛋白的短暂睫毛发生是成脂分化的基本特征。

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摘要

Bardet-Biedl syndrome (BBS) is an inherited ciliopathy generally associated with severe obesity, but the underlying mechanism remains hypothetical and is generally proposed to be of neuroen-docrine origin. In this study, we show that while the proliferating preadipocytes or mature adipocytes are nonciliated in culture, a typical primary cilium is present in differentiating preadipocytes. This transient cilium carries receptors for Wnt and Hedgehog pathways, linking this organelle to previously described regulatory pathways of adipogenesis. We also show that the BBS 10 and BBS 12 proteins are located within the basal body of this primary cilium and inhibition of their expression impairs ciliogenesis, activates the glycogen synthase kinase 3 pathway, and induces peroxisome proliferator-activated receptor nuclear accumulation, hence favoring adipogenesis. Moreover, adipocytes derived from BBS-patients' dermal fibroblasts in culture exhibit higher propensity for fat accumulation when compared to controls. This strongly suggests that a peripheral primary dysfunction of adipogenesis participates to the pathogenesis of obesity in BBS.
机译：Bardet-Biedl综合征（BBS）是一种遗传性纤毛病，通常与严重的肥胖症有关，但是其潜在机制仍是假设性的，通常被认为是神经元学说的。在这项研究中，我们表明，尽管培养中未增殖增殖前脂肪细胞或成熟脂肪细胞，但分化前脂肪细胞中存在典型的纤毛。这种瞬时纤毛携带Wnt和Hedgehog途径的受体，使该细胞器与先前描述的脂肪形成调节途径相关。我们还表明，BBS 10和BBS 12蛋白位于该初级纤毛的基体内，抑制它们的表达会损害纤毛发生，激活糖原合酶激酶3途径并诱导过氧化物酶体增殖物激活的受体核蓄积，因此有利于脂肪形成。此外，与对照组相比，来自BBS患者的真皮成纤维细胞的脂肪细胞在培养中表现出更高的脂肪蓄积倾向。这强烈表明，脂肪形成的外周原发性功能障碍参与了BBS中肥胖的发病机制。

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  • 作者

    Vincent Marion; Corinne Stoetzel; Dominique Schlicht; Nadia Messaddeq; Michael Koch; Elisabeth Flori; Jean Marc Danse; Jean-Louis Mandel; Helene Dollfus;

  • 作者单位

    Laboratoire Physiopathologie des Syndromes Rares Hereditaires, AVENIR-Inserm, EA3949, Faculte de Medecine de Strasbourg, Universite Louis Pasteur, 11 rue Humann, 67085 Strasbourg, France;

    Laboratoire Physiopathologie des Syndromes Rares Hereditaires, AVENIR-Inserm, EA3949, Faculte de Medecine de Strasbourg, Universite Louis Pasteur, 11 rue Humann, 67085 Strasbourg, France;

    Laboratoire Physiopathologie des Syndromes Rares Hereditaires, AVENIR-Inserm, EA3949, Faculte de Medecine de Strasbourg, Universite Louis Pasteur, 11 rue Humann, 67085 Strasbourg, France;

    Institut de Genetique et de Biologie Moleculaire et Cellulaire, Inserm U596, CNRS, UMR7104 Universite Louis Pasteur, Strasbourg, Illkirch, F-67400 France;

    Institut de Genetique et de Biologie Moleculaire et Cellulaire, Inserm U596, CNRS, UMR7104 Universite Louis Pasteur, Strasbourg, Illkirch, F-67400 France;

    Service de Cytogenetique, Hopitaux Universitaires de Strasbourg, Avenue Moliere, Strasbourg, France;

    Laboratoire Physiopathologie des Syndromes Rares Hereditaires, AVENIR-Inserm, EA3949, Faculte de Medecine de Strasbourg, Universite Louis Pasteur, 11 rue Humann, 67085 Strasbourg, France;

    Institut de Genetique et de Biologie Moleculaire et Cellulaire, Inserm U596, CNRS, UMR7104 Universite Louis Pasteur, Strasbourg, Illkirch, F-67400 France Chaire de Genetique Humaine, College de France, Illkirch, F-67400 France;

    Laboratoire Physiopathologie des Syndromes Rares Hereditaires, AVENIR-Inserm, EA3949, Faculte de Medecine de Strasbourg, Universite Louis Pasteur, 11 rue Humann, 67085 Strasbourg, France;

  • 收录信息 美国《科学引文索引》(SCI);美国《生物学医学文摘》(MEDLINE);美国《化学文摘》(CA);
  • 原文格式 PDF
  • 正文语种 eng
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  • 关键词

    adipogenesis; primary cilium; ciliopathy; obesity;

    机译：脂肪形成;初级纤毛;纤毛病;肥胖;

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