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Elsevier电子期刊(33)

在“Elsevier电子期刊”中，命中：33条，耗时：小于0.01 秒

在所有数据库中总计命中：33条

1.Eleven novel mutations of the BCKDHA, BCKDHB and DBT genes associated with maple syrup urine disease in the Chinese population: Report on eight cases

作者：Xiyuan Li^a ; Yuan Ding^a ; Yupeng Liu^a ; Yanyan Ma^a ; Jinqing Song^a ; Qiao Wang^a ; Mengqiu Li^b ; Yaping Qin^b ; Yanling Yang^a ; ^{yanlingy@vip.sina.com" class="auth_mail" title="E-mail the corresponding author} ; ^{organic.acid@126.com" class="auth_mail" title="E-mail the corresponding author}

关键词：Maple syrup urine disease ; Branched chain amino acids ; BCKDHA gene ; BCKDHB gene ; DBT gene ; Prenatal diagnosis

刊名：European Journal of Medical Genetics

出版年：2015

2.Identification of a novel BCKDHA deletion causing maple syrup urine disease

作者：Sara Bremer^a ; ^{sbremer@ous-hf.no" class="auth_mail" title="E-mail the corresponding author} ; Yngve Thomas Bliksrud^a ; Helge Rootwelt^a ; Berit Woldseth^a ; Trine Tangeraas^b ; Ingjerd Sæ ; ves^b ; Sara Sofie Viksmoen Watle^b

关键词：Maple syrup urine disease ; BCKDHA ; branched-chain amino acids ; newborn screening

刊名：Meta Gene

出版年：2016

3.Molecular analysis of maple syrup urine disease in Jordanian families

关键词：Maple syrup urine disease ; Branched-chain α-ketoacid dehydrogenase complex ; BCKDHA ; DBT ; Jordan

刊名：Meta Gene

出版年：2016

4.Identification of six novel mutations in Iranian patients with maple syrup urine disease and their in silico analysis‿

作者：Maryam Abiri^a ; ^b ; Razieh Karamzadeh^c ; ^d ; Morteza Karimipoor^b ; Shirin Ghadami^b ; Mohammad Reza Alaei^e ; Samira Dabagh Bagheri^f ; Hamideh Bagherian^f ; Aria Setoodeh^g ; Mohammad Reza Noori-Daloii^a ; Sirous Zeinali^b ; ^f ; ^{zeinali@kawsar.ir" class="auth_mail" title="E-mail the corresponding author}

关键词：MSUD ; BCKDHA gene ; Iran ; Mutation analysis

刊名：Mutation Research/Fundamental and Molecular Mechanisms of Mutagenesis

出版年：2016

5.Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment

作者：I. Bravo-Alonso^a ; A. Oyarzabal^a ; M. Sá ; nchez-Aragó ; ^b ; M.T. Rejas^c ; B. Merinero^a ; A. Garcí ; a-Cazorla^d ; R. Artuch^e ; M. Ugarte^a ; P. Rodrí ; guez-Pombo^a ; ^{mprodriguez@cbm.csic.es" class="auth_mail" title="E-mail the corresponding author}

关键词：BCKDK interference ; Branched-chain α-ketoacid dehydrogenase ; Bioenergetics profile ; Unrestrained branched-chain amino acids catabolism

刊名：Data in Brief

出版年：2016

6.Branched-chain amino acid restriction in Zucker-fatty rats improves muscle insulin sensitivity by enhancing efficiency of fatty acid oxidation and acyl-glycine export

作者：Phillip J. White¹ ; ³ ; Amanda L. Lapworth² ; ³ ; Jie An¹ ; Liping Wang¹ ; Robert W. McGarrah¹ ; Robert D. Stevens¹ ; Olga Ilkayeva¹ ; Tabitha George¹ ; Michael J. Muehlbauer¹ ; James R. Bain¹ ; Jeff K. Trimmer² ; M. Julia Brosnan² ; Timothy P. Rolph² ; Christopher B. Newgard¹ ; ^{Chris.Newgard@duke.edu" class="auth_mail" title="E-mail the corresponding author}

关键词：Obesity ; BCAA ; Insulin sensitivity ; Metabolism

刊名：Molecular Metabolism

出版年：2016

7.l-Carnitine supplementation decreases DNA damage in treated MSUD patients

作者：Caroline Paula Mescka^c ; ^{carolmescka@yahoo.com.br" class="auth_mail" title="E-mail the corresponding author} ; Gilian Guerreiro^a ; Tatiane Hammerschmidt^a ; Jé ; ssica Faverzani^a ; Daniella de Moura Coelho^b ; Vanusa Mandredini^e ; Carlos Alberto Yasin Wayhs^d ; Moacir Wajner^b ; ^c ; Carlos Severo Dutra-Filho^c ; Carmen Regla Vargas^a ; ^b ; ^c ; ^d

关键词：MSUD ; maple syrup urine disease ; BCAAs ; branched-chain amino acids ; l-car ; l-carnitine ; DI ; damage index ; MDA ; malondialdehyde ; BCKDH ; branched-chain α-keto acid dehydrogenase ; BCKA ; branched-chain α-keto acids ; KIC ; α-ketoisocaproate ; Leu ; leucine ; Ile ; isoleucine ; Val ; valine

刊名：Mutation Research/Fundamental and Molecular Mechanisms of Mutagenesis

出版年：2015

8.Stimulation of rat liver branched-chain alpha-keto acid dehydrogenase activity by low doses of bezafibrate

作者：Malgorzata Knapik-Czajka ; ^{mfknapik@cyf-kr.edu.pl}

关键词：Bezafibrate ; Branched-chain alpha-ketoacid dehydrogenase (BCKDH) ; Leucine

刊名：Toxicology

出版年：2013

9.Two novel mutations in the BCKDHB gene (R170H, Q346R) cause the classic form of maple syrup urine disease (MSUD)

作者：Yue-Ping Wang^a ; ^{wangyp@sj-hospital.org} ; Man-Long Qi^a ; Ting-Ting Li^a ; Yun-Jing Zhao^b

关键词：MSUD ; maple syrup urine disease ; BCKD ; branched-chain 2-keto acid dehydrogenase ; BCAA ; branched-chain amino acid ; BCKAs ; branched-chain ¦Á-ketoacid ; E1 ; branched-chain ¦Á-keto-acid decarboxylase ; E2 ; a dihydrolipoyl transacylase ; E3 ; a dihydrolipoamide de

刊名：Gene

出版年：2012

10.Regulation of hepatic branched-chain α-keto acid dehydrogenase kinase in a rat model for type 2 diabetes mellitus at different stages of the disease

作者：Masao Doisaki ; Yoshiaki Katano ; Isao Nakano ; Yoshiki Hirooka ; Akihiro Itoh ; Masatoshi Ishigami ; Kazuhiko Hayashi ; Hidemi Goto ; Yuko Fujita ; Yoshihiro Kadota ; Yasuyuki Kitaura ; Gustavo Bajotto ; Shunsuk

关键词：Branched-chain amino acid (BCAA) ; Type 2 diabetes mellitus ; Branched-chain α ; -keto acid dehydrogenase (BCKDH) complex ; BCKDH kinase ; Zucker diabetic fatty (ZDF) rat

刊名：Biochemical and Biophysical Research Communications

出版年：2010

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