نتایج جستجو برای: mcardle cells and vldl

تعداد نتایج: 17076115  

Journal: :Human mutation 2007
Juan C Rubio Ines Garcia-Consuegra Gisela Nogales-Gadea Alberto Blazquez Ana Cabello Alejandro Lucia Antoni L Andreu Joaquin Arenas Miguel A Martin

McArdle disease is a metabolic myopathy due to molecular defects in the myophosphorylase gene (PYGM), usually diagnosed in muscle biopsy. The aims of this study were to characterize genetically a large series of patients and to establish a protocol of molecular diagnosis on blood samples. We studied 55 Spanish unrelated patients with McArdle disease. Screening for the three more frequent mutati...

Journal: :Swiss Medical Forum ‒ Schweizerisches Medizin-Forum 2015

Journal: :Proceedings of the National Academy of Sciences 1981

Journal: :Arquivos de Neuro-Psiquiatria 1980

Journal: :European Heart Journal 2018

2010
Gisela Nogales-Gadea Emma Mormeneo Inés García-Consuegra Juan C. Rubio Anna Orozco Joaquin Arenas Miguel A. Martín Alejandro Lucia Anna M. Gómez-Foix Ramon Martí Antoni L. Andreu

BACKGROUND Mutations in the PYGM gene encoding skeletal muscle glycogen phosphorylase (GP) cause a metabolic disorder known as McArdle's disease. Previous studies in muscle biopsies and cultured muscle cells from McArdle patients have shown that PYGM mutations abolish GP activity in skeletal muscle, but that the enzyme activity reappears when muscle cells are in culture. The identification of t...

Journal: :Arteriosclerosis, Thrombosis, and Vascular Biology 1995

2005
O. Stein

Thioglycolate-elicited mouse peritoneal macrophages were Incubated for 24 hours in serum-free DulbeccoVogt medium containing 0.5% fatty acid-poor bovine serum albumin. This conditioned medium, designated MP medium, was used for experiments with bovine aortic smooth muscle cells (SMCs) or human skin flbroblasts (HSFs). Dulbecco-Vogt medium of the same albumin content but without macrophages serv...

Journal: :Science translational medicine 2016
Gissette Reyes-Soffer Byoung Moon Antonio Hernandez-Ono Marija Dionizovik-Dimanovski Jhonsua Jimenez Joseph Obunike Tiffany Thomas Colleen Ngai Nelson Fontanez Daniel S Donovan Wahida Karmally Stephen Holleran Rajasekhar Ramakrishnan Robert S Mittleman Henry N Ginsberg

Mipomersen is a 20mer antisense oligonucleotide (ASO) that inhibits apolipoprotein B (apoB) synthesis; its low-density lipoprotein (LDL)-lowering effects should therefore result from reduced secretion of very-low-density lipoprotein (VLDL). We enrolled 17 healthy volunteers who received placebo injections weekly for 3 weeks followed by mipomersen weekly for 7 to 9 weeks. Stable isotopes were us...

Journal: :The EMBO journal 2007
Peter Michaely Zhenze Zhao Wei-Ping Li Rita Garuti Lily J Huang Helen H Hobbs Jonathan C Cohen

The low-density lipoprotein (LDL) receptor (LDLR) binds to and internalizes lipoproteins that contain apolipoproteinB100 (apoB100) or apolipoproteinE (apoE). Internalization of the apoB100 lipoprotein ligand, LDL, requires the FDNPVY(807) sequence on the LDLR cytoplasmic domain, which binds to the endocytic machinery of coated pits. We show here that inactivation of the FDNPVY(807) sequence by ...

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