نتایج جستجو برای: seipin

تعداد نتایج: 156  

Journal: :Biochemical Society transactions 2005
J Capeau J Magré O Lascols M Caron V Béréziat C Vigouroux J P Bastard

Human lipodystrophies represent a group of diseases characterized by altered body fat amount and/or repartition and major metabolic alterations with insulin resistance leading to diabetic complications and increased cardiovascular and hepatic risk. Genetic forms of lipodystrophies are rare. Congenital generalized lipodystrophy or Berardinelli-Seip syndrome, autosomal recessive, is characterized...

Journal: :Brain : a journal of neurology 2004
Joy Irobi Peter Van den Bergh Luciano Merlini Christine Verellen Lionel Van Maldergem Ines Dierick Nathalie Verpoorten Albena Jordanova Christian Windpassinger Els De Vriendt Veerle Van Gerwen Michaela Auer-Grumbach Klaus Wagner Vincent Timmerman Peter De Jonghe

Silver syndrome is a rare autosomal dominant neurodegenerative disorder characterized by marked amyotrophy and weakness of small hand muscles and spasticity in the lower limbs. The locus for Silver syndrome (SPG17) was assigned to a 13 cM region on chromosome 11q12-q14 in a single large pedigree. We recently found heterozygous mutations in the Berardinelli-Seip congenital lipodystrophy (BSCL2, ...

Journal: :Endocrine development 2010
Jacqueline Capeau Jocelyne Magré Martine Caron-Debarle Claire Lagathu Bénédicte Antoine Véronique Béréziat Olivier Lascols Jean-Philippe Bastard Corinne Vigouroux

Human lipodystrophies represent a heterogeneous group of diseases characterized by generalized or partial fat loss, with fat hypertrophy in other depots when partial. Insulin resistance, dyslipidemia and diabetes are generally associated, leading to early complications. Genetic forms are uncommon: recessive generalized congenital lipodystrophies result in most cases from mutations in the genes ...

Journal: :Journal of lipid research 2011
Xin-Gui Peng Shenghong Ju Yujiao Qin Fang Fang Xin Cui George Liu Yicheng Ni Gao-Jun Teng

We evaluated dual-echo Dixon in-phase and out-of-phase (IP-OP), chemical shift imaging (CSI), and (1)H MRS (hydrogen MR spectroscopy) in estimating fat content (FC) in phantoms and in livers of mice. Phantoms were made according to the volume percentage of fat ranging from 0% to 100%. The three MR methods were performed to measure FC in phantoms and in livers of obese leptin-deficient (ob/ob), ...

Journal: :Diabetes 2008
Victoria A. Payne Neil Grimsey Antoinette Tuthill Sam Virtue Sarah L. Gray Edoardo Dalla Nora Robert K. Semple Stephen O'Rahilly Justin J. Rochford

OBJECTIVE Berardinelli-Seip congenital lipodystrophy type 2 (BSCL2) is a recessive disorder featuring near complete absence of adipose tissue. Remarkably, although the causative gene, BSCL2, has been known for several years, its molecular function and its role in adipose tissue development have not been elucidated. Therefore, we examined whether BSCL2 is involved in the regulation of adipocyte ...

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