نتایج جستجو برای: bscl2 gene

تعداد نتایج: 1141398  

Journal: :Human molecular genetics 2013
Maarit Hölttä-Vuori Veijo T Salo Yuki Ohsaki Maximiliano L Suster Elina Ikonen

Mutations affecting the N-glycosylation site in Berardinelli-Seip lipodystrophy (BSCL)-associated gene BSCL2/seipin lead to a dominantly inherited spastic paraplegia termed seipinopathy. While the loss of function of seipin leads to severe congenital lipodystrophy, the effects of seipin N-glycosylation mutations on lipid balance in the nervous system are unknown. In this study, we show that exp...

Journal: :American journal of physiology. Endocrinology and metabolism 2013
Xin-Gui Peng Shenghong Ju Fang Fang Yu Wang Ke Fang Xin Cui George Liu Peng Li Hui Mao Gao-Jun Teng

Brown adipose tissue (BAT) plays a key role in thermogenesis to protect the body from cold and obesity. White adipose tissue (WAT) stores excess energy in the form of triglycerides. To better understand the genetic effect on regulation of WAT and BAT, we investigated the fat fraction (FF) in two types of adipose tissues in ob/ob, human BSCL2/seipin gene knockout (SKO), Fsp27 gene knockout (Fsp2...

2012
Xin-Gui Peng Shenghong Ju Fang Fang Yu Wang Ke Fang Xin Cui George Liu Peng Li Hui Mao Gao-Jun Teng

Xin-Gui Peng, Shenghong Ju, Fang Fang, Yu Wang, Ke Fang, Xin Cui, George Liu, Peng Li, Hui Mao, and Gao-Jun Teng Jiangsu Key Laboratory of Molecular and Functional Imaging, Department of Radiology, Zhongda Hospital, Medical School, Southeast University, Nanjing, China; Bruker BioSpin, Beijing, China; Institute of Cardiovascular Sciences and Key Laboratory of Molecular Cardiovascular Sciences, M...

2018
Mauricio A Cuello Sumie Kato Francisca Liberona

To investigate whether specific obesity/metabolism-related gene expression patterns affect the survival of patients with ovarian cancer. Clinical and genomic data of 590 samples from the high-grade ovarian serous carcinoma (HGOSC) study of The Cancer Genome Atlas (TCGA) and 91 samples from the Australian Ovarian Cancer Study were downloaded from the International Cancer Genome Consortium (ICGC)...

Journal: :Diagnostic and interventional radiology 2017
Canan Altay Mustafa Seçil Tevfik Demir Tahir Atik Gülçin Akıncı Nilüfer Özdemir Kutbay Ela Keskin Temeloğlu Ilgın Yıldırım Şimşir Seçil Özışık Leyla Demir Erdal Eren Emine Burçin Tuna Hasibe Aytaç Hüseyin Onay Barış Akıncı

PURPOSE We aimed to investigate residual adipose tissue with whole-body magnetic resonance imaging to differentiate between subtypes of lipodystrophy. METHODS A total of 32 patients (12 with congenital generalized lipodystrophy [CGL], 1 with acquired generalized lipodystrophy [AGL], 12 with familial partial lipodystrophy [FPLD], and 7 with acquired partial lipodystrophy [APL]) were included. ...

Journal: :Human molecular genetics 2012
Daisuke Ito Takuya Yagi Masahito Ikawa Norihiro Suzuki

Gain-of-toxic mutations in the N-glycosylation motif of the seipin/BSCL2 gene (namely, the N88S and S90L mutations) cause autosomal dominant motor neuron diseases, termed 'seipinopathy'. Expressed mutant seipin is improperly folded and accumulates in the endoplasmic reticulum (ER), leading to an unfolded protein response (UPR). Furthermore, cells expressing mutant seipin contain unique cytoplas...

پایان نامه :وزارت علوم، تحقیقات و فناوری - دانشگاه تربیت مدرس - دانشکده علوم پزشکی 1391

مطالعات انجام شده روی مکانیسمهای تکوینی و تکاملی گلبولهای قرمز منجر به دستیابی بشر به مفاهیم پایه و مهمی در ارتباط با مکانیسمهای عمومی تنظیم بیان ژن وشکل گیری بافتها شده است. تمایز اختصاصی به رده ارتیروئید و هر رده دیگری، شدیداً وابسته به تنظیم در سطح بیان ژن و فاکتورهای کنترلی خاص نظیر سیتوکین ها، فاکتورهای نسخه برداری ویژه، عناصر کنترل کننده چرخه سلولی، تکثیر،آپوپتوز و عناصر سیگنالینگ داخل سلو...

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