نتایج جستجو برای: seip lypodystrophy

تعداد نتایج: 181  

Journal: :Extensão em Ação 2023

A Síndrome de Berardinelli-Seip (SBS) é uma rara doença genética autossômica recessiva, alta prevalência no Rio Grande do Norte (RN), Brasil. O presente trabalho objetivou relatar a experiência dos acadêmicos enfermagem, nutrição, psicologia e fisioterapia da Faculdade Ciências Saúde Trairi (FACISA) durante ações multiprofissionais que englobavam pais pessoas com SBS RN. Nesse projeto, foram re...

Journal: :Journal of medical genetics 2002
L Van Maldergem J Magré T E Khallouf T Gedde-Dahl M Delépine O Trygstad E Seemanova T Stephenson C S Albott F Bonnici V R Panz J L Medina P Bogalho F Huet S Savasta A Verloes J J Robert H Loret M De Kerdanet N Tubiana-Rufi A Mégarbané J Maassen M Polak D Lacombe C R Kahn E L Silveira F H D'Abronzo F Grigorescu M Lathrop J Capeau S O'Rahilly

Generalised lipodystrophy of the Berardinelli-Seip type (BSCL) is a rare autosomal recessive human disorder with severe adverse metabolic consequences. A gene on chromosome 9 (BSCL1) has recently been identified, predominantly in African-American families. More recently, mutations in a previously undescribed gene of unknown function (BSCL2) on chromosome 11, termed seipin, have been found to be...

Journal: :The Journal of Cell Biology 2008
Weihua Fei Guanghou Shui Bruno Gaeta Ximing Du Lars Kuerschner Peng Li Andrew J. Brown Markus R. Wenk Robert G. Parton Hongyuan Yang

Lipid droplets (LDs) are emerging cellular organelles that are of crucial importance in cell biology and human diseases. In this study, we present our screen of approximately 4,700 Saccharomyces cerevisiae mutants for abnormalities in the number and morphology of LDs; we identify 17 fld (few LDs) and 116 mld (many LDs) mutants. One of the fld mutants (fld1) is caused by the deletion of YLR404W,...

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