نتایج جستجو برای: bcaa

تعداد نتایج: 596  

Journal: :Science 2016
Jared R Mayers Margaret E Torrence Laura V Danai Thales Papagiannakopoulos Shawn M Davidson Matthew R Bauer Allison N Lau Brian W Ji Purushottam D Dixit Aaron M Hosios Alexander Muir Christopher R Chin Elizaveta Freinkman Tyler Jacks Brian M Wolpin Dennis Vitkup Matthew G Vander Heiden

Tumor genetics guides patient selection for many new therapies, and cell culture studies have demonstrated that specific mutations can promote metabolic phenotypes. However, whether tissue context defines cancer dependence on specific metabolic pathways is unknown. Kras activation and Trp53 deletion in the pancreas or the lung result in pancreatic ductal adenocarinoma (PDAC) or non-small cell l...

2016
Courtney R. Green Martina Wallace Ajit S. Divakaruni Susan A. Phillips Anne N. Murphy Theodore P. Ciaraldi Christian M. Metallo

Adipose tissue plays important roles in regulating carbohydrate and lipid homeostasis, but less is known about the regulation of amino acid metabolism in adipocytes. Here we applied isotope tracing to pre-adipocytes and differentiated adipocytes to quantify the contributions of different substrates to tricarboxylic acid (TCA) metabolism and lipogenesis. In contrast to proliferating cells, which...

2013
HITOSHI YOSHIJI RYUICHI NOGUCHI TADASHI NAMISAKI KEI MORIYA MITSUTERU KITADE YOSUKE AIHARA AKITOSHI DOUHARA JUNICHI YAMAO MASAO FUJIMOTO MASAHISA TOYOHARA AKIRA MITORO MASAYOSHI SAWAI MOTOYUKI YOSHIDA CHIE MORIOKA MASAKAZU UEJIMA MASAHITO UEMURA HIROSHI FUKUI

Branched-chain amino acids (BCAAs) reportedly inhibit the incidence of hepatocellular carcinoma (HCC) in patients with liver cirrhosis and obesity that is frequently associated with insulin resistance (IR). We previously reported that BCAAs exert a chemopreventive effect against HCC under IR conditions in rats. The aim of the present study was to examine the effect of BCAAs on the cumulative re...

2011
Andrej Kochevenko Alisdair R. Fernie

Previous studies of the genetic architecture of fruit metabolic composition have allowed us to identify four strongly conserved co-ordinate quantitative trait loci (QTL) for the branched-chain amino acids (BCAAs). This study has been extended here to encompass the other 23 enzymes described to be involved in the pathways of BCAA synthesis and degradation. On coarse mapping the chromosomal locat...

Journal: :The Journal of nutrition 2006
David T Chuang Jacinta L Chuang R Max Wynn

Genetic disorders of BCAA metabolism produce amino acidopathies and various forms of organic aciduria with severe clinical consequences. A metabolic block in the oxidative decarboxylation of BCAA caused by mutations in the mitochondrial branched-chain alpha-keto acid dehydrogenase complex (BCKDC) results in Maple Syrup Urine Disease (MSUD) or branched-chain ketoaciduria. There are presently fiv...

2014
NAMIKO AMANO KOTARO KITAYA SAGIRI TAGUCHI MIYAKO FUNABIKI YOSHIHIRO TADA TERUMI HAYASHI YOSHITAKA NAKAMURA

The aim of this study was to compare the branched-chain amino acid (BCAA) and tyrosine concentration in the follicular fluid of infertile women with and without ovarian hyperstimulation syndrome (OHSS) in an in vitro fertilization program combined with controlled ovarian stimulation. Follicular fluid was aspirated during oocyte retrieval from 20 infertile patients who developed moderate-to-seve...

Journal: :The Journal of clinical investigation 2009
Gang Lu Haipeng Sun Pengxiang She Ji-Youn Youn Sarah Warburton Peipei Ping Thomas M Vondriska Hua Cai Christopher J Lynch Yibin Wang

The branched-chain amino acids (BCAA) are essential amino acids required for protein homeostasis, energy balance, and nutrient signaling. In individuals with deficiencies in BCAA, these amino acids can be preserved through inhibition of the branched-chain-alpha-ketoacid dehydrogenase (BCKD) complex, the rate-limiting step in their metabolism. BCKD is inhibited by phosphorylation of its E1alpha ...

2005
David T. Chuang Jacinta L. Chuang Max Wynn

Genetic disorders of BCAA metabolism produce amino acidopathies and various forms of organic aciduria with severe clinical consequences. A metabolic block in the oxidative decarboxylation of BCAA caused by mutations in the mitochondrial branched-chain a-keto acid dehydrogenase complex (BCKDC) results in Maple Syrup Urine Disease (MSUD) or branched-chain ketoaciduria. There are presently five kn...

Journal: :Cell 2016
Dora C. Tărlungeanu Elena Deliu Christoph P. Dotter Majdi Kara Philipp Christoph Janiesch Mariafrancesca Scalise Michele Galluccio Mateja Tesulov Emanuela Morelli Fatma Mujgan Sonmez Kaya Bilguvar Ryuichi Ohgaki Yoshikatsu Kanai Anide Johansen Seham Esharif Tawfeg Ben-Omran Meral Topcu Avner Schlessinger Cesare Indiveri Kent E. Duncan Ahmet Okay Caglayan Murat Gunel Joseph G. Gleeson Gaia Novarino

Autism spectrum disorders (ASD) are a group of genetic disorders often overlapping with other neurological conditions. We previously described abnormalities in the branched-chain amino acid (BCAA) catabolic pathway as a cause of ASD. Here, we show that the solute carrier transporter 7a5 (SLC7A5), a large neutral amino acid transporter localized at the blood brain barrier (BBB), has an essential...

Journal: :PLoS ONE 2007
Fanny Mochel Perrine Charles François Seguin Julie Barritault Christiane Coussieu Laurence Perin Yves Le Bouc Christiane Gervais Guislaine Carcelain Anne Vassault Josué Feingold Daniel Rabier Alexandra Durr

Huntington disease (HD) is a fatal neurodegenerative disorder, with no effective treatment. The pathogenic mechanisms underlying HD has not been elucidated, but weight loss, associated with chorea and cognitive decline, is a characteristic feature of the disease that is accessible to investigation. We, therefore, performed a multiparametric study exploring body weight and the mechanisms of its ...

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