نتایج جستجو برای: erlin2

تعداد نتایج: 18  

Journal: :Human molecular genetics 2011
Yeşerin Yıldırım Elif Kocasoy Orhan Sibel Aylin Ugur Iseri Piraye Serdaroglu-Oflazer Bülent Kara Seyhun Solakoğlu Aslıhan Tolun

We present a family afflicted with a novel autosomal recessive disease characterized by progressive intellectual disability, motor dysfunction and multiple joint contractures. No pathology was found by cranial imaging, electromyography and muscle biopsy, but electron microscopy in leukocytes revealed large vacuoles containing flocculent material. We mapped the disease gene by SNP genome scan an...

2015
Bo Yang Mingliang Qu Rengang Wang Jon E Chatterton Xiao-Bo Liu Bing Zhu Sonoko Narisawa Jose Luis Millan Nobuki Nakanishi Kathryn Swoboda Stuart A Lipton Dongxian Zhang Freda Miller

Hitherto, membralin has been a protein of unknown function. Here, we show that membralin mutant mice manifest a severe and early-onset motor neuron disease in an autosomal recessive manner, dying by postnatal day 5-6. Selective death of lower motor neurons, including those innervating the limbs, intercostal muscles, and diaphragm, is predominantly responsible for this fatal phenotype. Neural ex...

2010
Quan Yang Gang Liu Aliccia Bollig-Fischer Craig N. Giroux Stephen P. Ethier

nloaded plification of the 8p11-12 region has been found in about 15% of human breast cancers and is associated oor prognosis. Earlier, we used genomic analysis of copy number and gene expression to perform a d analysis of the 8p11-12 amplicon to identify candidate oncogenes in breast cancer. We identified didate genes and provided evidence that three genes, namely, LSM-1, TC-1, and BAG4, have ...

Journal: :Journal of cell science 2006
Duncan T Browman Mary E Resek Laura D Zajchowski Stephen M Robbins

Our laboratory was interested in characterizing the molecular composition of non-caveolar lipid rafts. Thus, we generated monoclonal antibodies to lipid raft proteins of human myelomonocytic cells. Two of these proteins, KE04p and C8orf2, were found to be highly enriched in the detergent-insoluble, buoyant fraction of sucrose gradients in a cholesterol-dependent manner. They contain an evolutio...

2011
Youngah Jo Peter V. Sguigna Russell A. DeBose-Boyd

The endoplasmic reticulum (ER)-associated degradation (ERAD) pathway in the yeast Saccharomyces cerevisiae is mediated by two membrane-bound ubiquitin ligases, Doa10 and Hrd1. These enzymes are found in distinct multiprotein complexes that allow them to recognize and target a variety of substrates for proteasomal degradation. Although multiprotein complexes containing mammalian ERAD ubiquitin l...

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