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HACD1,a regulator of membrane composition and fluidity,promotes myoblast fusion and skeletal muscle growth

Journal of Molecular Cell Biology 2015年第5期7卷 429-440页

作者： Jordan Blondelle Yusuke Ohno Vincent Gache Stephane Guyot Sebastien Storck Nicolas Blanchard-Gutton Ines Barthelemy Gemma Walmsley Anaelle Rahier Stephanie Gadin Marie Maurer laurent Guillaud Alexandre Prola Arnaud Ferry Genevieve Aubin-Houzelstein Jean Demarquoy Frederic Relaix Richard JPiercy Stephane Blot Akio Kihara laurent tiret Fanny Pilot-Storck Inserm IMRB U955-E1094000 CreteilFrance Universite Paris-Est Ecole nationale veterinaire d’Alfort(EnvA)94700 Maisons-AlfortFrance Universite Paris-Est Creteil Faculte de medecine94000 CreteilFrance Laboratory of Biochemistry Faculty of Pharmaceutical SciencesHokkaido UniversitySapporo 060-0812Japan Universite de Bourgogne UMR A 02.102 PAM-EPMBAgroSup Dijon21000 DijonFrance Institut Necker-Enfants Malades INSERM U1151-CNRS UMR 8253Sorbonne Paris CiteUniversite Paris DescartesFaculte de Medecine-Site Broussais75015 ParisFrance Comparative Neuromuscular Disease Laboratory Department of Clinical Sciences and ServicesRoyal Veterinary CollegeLondon NW10TUUK Therapie des maladies du muscle strie INSERM U974-CNRS UMR7215-UPMC UM76-Institut de Myologie Universite Pierre et Marie Curie-Universite Paris Descartes75000 ParisFrance Universite de Bourgogne Faculte des Sciences GabrielBio-PeroxIL21000 DijonFrance

The reduced diameter of skeletal myofibres is a hallmark of several congenital myopathies,yet the underlying cellular and molecular mechanisms remain *** this study,we investigate the role of HACD1/PTPLA,which is involved in the elongation of the very long chain fatty acids,in muscle fibre *** humans and dogs,HACD1 deficiency leads to a congenital myopathy with fibre size disproportion associated with a generalized *** of HACD1-deficient Labradors,Hacd1-knockout mice,and Hacd1-deficient myoblasts,we provide evidence that HACD1 promotes myoblast fusion during muscle development and *** further demonstrate that in normal differentiating myoblasts,expression of the catalytically active HACD1 isoform,which is encoded by a muscle-enriched splice variant,yields decreased lysophosphatidylcholine content,a potent inhibitor of myoblast fusion,and increased concentrations of≥C18 and monounsaturated fatty acids of *** lipid modifications correlate with a reduction in plasma membrane *** conclusion,we propose that fusion impairment constitutes a novel,non-exclusive pathological mechanism operating in congenital myopathies and reveal that HACD1 is a key regulator of a lipid-dependent muscle fibre growth mechanism.

关键词： centronuclear myopathy LPC MUFA PTPLA VLCFA

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Disease activity and cancer risk in inflammatory bowel disease associated with primary sclerosing cholangitis

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World Journal of Gastroenterology 2008年第22期14卷 3497-3503页

作者： Harry Sokol Jacques Cosnes Olivier Chazouilleres laurent Beaugerie Emmanuel tiret Raoul Poupon Philippe Seksik Gastroenterology and Nutrition Department Hpital Saint-Antoine Université Pierre et Marie Curie-Paris6 AP-HP Paris 75571 France Hepatology Depart-ment Hpital Saint-Antoine Université Pierre et Marie Curie-Paris6 AP-HP Paris 75571 France Department of Abdominal Surgery Hpital Saint-Antoine Université Pierre et Marie Curie-Paris6 AP-HP Paris 75571 France

AIM: To investigate the phenotype of inflammatory bowel disease associated with primary sclerosing cholangitis (PSC-IBD). METHODS: Data from 75 PSC-IBD patients evaluated in our tertiary center between 1963 and 2006 were collected and compared to 150 IBD patients without PSC, matched for sex, birth date, IBD diagnosis date and initial disease location regarding ileal, different colonic segments, and rectum, respectively. RESULTS: While PSC-IBD patients received more 5-aminosalicylates (8.7 years/patient vs 2.9 years/ patient, P < 0.001), they required less immuno-suppressors (24% vs 46% at 10 years; P < 0.001) and less intestinal resection (10% vs 44% at 10 years, P < 0.001). The 25-year cumulative rate of colectomy was 25.1% in PSC-IBD and 37.3% in controls (P = 0.004). The 25-year cumulative rate of colorectal cancer was 23.4% in PSC-IBD vs 0% in controls (P = 0.002). PSC was the only independent risk factor for the development of colorectal cancer (OR = 10.8; 95% CI, 3.7-31.3). Overall survival rate without liver transplantation was reduced in PSC-IBD patients (67% vs 91% in controls at 25 years, P = 0.001).CONCLUSION: This study confirms that patients with PSC-IBD have a particular disease phenotype independent of the initial disease location. Although their disease is less active and they use more 5-aminosalicylates, they present a higher risk of colorectal cancer.

关键词： Primary sclerosing cholangitis Inflammatory bowel disease Colorectal cancer Ulcerative colitis Crohn's disease

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