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Wiley电子期刊(3)	SpringerLink电子期刊(140)	Elsevier电子期刊(57)
Springer电子图书(1)	ProQuest学位论文(1)

在“Elsevier电子期刊”中，命中：57条，耗时：0.04001 秒

在所有数据库中总计命中：202条

1.Efficacy of bezafibrate on fibroblasts of glutaric acidemia type II patients evaluated using an in vitro probe acylcarnitine assay

作者：Kenji Yamada^a ; ^{k-yamada@med.shimane-u.ac.jp} ; Hironori Kobayashi^a ; Ryosuke Bo^a ; ^b ; Jamiyan Purevsuren^a ; Yuichi Mushimoto^a ; Tomoo Takahashi^a ; Yuki Hasegawa^a ; Takeshi Taketani^a ; Seiji Fukuda^a ; Seiji Yamaguchi^a

关键词：Bezafibrate ; Glutaric acidemia type II ; Multiple acyl-CoA dehydrogenase deficiency ; In vitro probe assay ; Fatty acid oxidation disorders

刊名：Brain and Development

出版年：2017

2.Severe sensory neuropathy in patients with adult-onset multiple acyl-CoA dehydrogenase deficiency

作者：Zhaoxia Wang^a ; ¹ ; Daojun Hong^b ; ¹ ; Wei Zhang^a ; Wurong Li^c ; Xin Shi^a ; Danhua Zhao^a ; Xu Yang^d ; He Lv^a ; Yun Yuan^a ; ^{yuanyun2002@sohu.com" class="auth_mail" title="E-mail the corresponding author}

关键词：Multiple Acyl-CoA dehydrogenase deficiency ; Sensory neuropathy ; ETFDH ; Phenotype

刊名：Neuromuscular Disorders

出版年：2016

3.Quantification of hypoglycin A as butyl ester

作者：Johannes Sander^a ; ^{j.sander@metabscreen.de" class="auth_mail" title="E-mail the corresponding author} ; Michael Terhardt^a ; Stefanie Sander^a ; Nils Janzen^a ; ^b

关键词：AM ; atypical myopathy in horses ; HGA ; hypoglycin A (l-α-amino-methylenecyclopropyl propionic acid) ; MADD ; multiple acyl carnitine dehydrogenase deficiency ; MCPA ; methylenecyclopropylacetic acid

刊名：Journal of Chromatography B, Analytical Technologies in the Biomedical and Life Sciences

出版年：2016

4.Clinical, biochemical and molecular investigation of adult-onset glutaric acidemia type II: Characteristics in comparison with pediatric cases

作者：Kenji Yamada^a ; ^{k-yamada@med.shimane-u.ac.jp" class="auth_mail" title="E-mail the corresponding author} ; Hironori Kobayashi^a ; Ryosuke Bo^a ; Tomoo Takahashi^a ; Jamiyan Purevsuren^a ; Yuki Hasegawa^a ; Takeshi Taketani^a ; Seiji Fukuda^a ; Takuya Ohkubo^b ; Takanori Yokota^b ; Mutsufusa Watanabe^c ; Taiji Tsunemi^d ; Hidehiro Mizusawa^e ; Hiroshi Takuma^f ; Ayako Shioya^f ; Akiko Ishii^f ; Akira Tamaoka^f ; Yosuke Shigematsu^g ; Hideo Sugie^h ; Seiji Yamaguchi^a

关键词：Multiple acyl-CoA dehydrogenase deficiency (glutaric acidemia type II) ; Adult onset ; Myopathy ; Serum acylcarnitine ; Immunoblotting ; In vitro probe acylcarnitine assay

刊名：Brain and Development

出版年：2016

5.Equine atypical myopathy: A metabolic study

作者：R. Karlí ; ková ; ^a ; ¹ ; J. &Scaron ; iroká ; ^a ; ¹ ; ^{sirokajitka@gmail.com" class="auth_mail" title="E-mail the corresponding author} ; P. Jahn^b ; D. Friedecký ; ^a ; ^c ; A. Gardlo^a ; ^d ; H. Janečková ; ^a ; ^c ; F. Hrdinová ; ^b ; Z. Drá ; bková ; ^b ; T. Adam^a ; ^c

关键词：Atypical myopathy ; Horse ; Hypoglycin A ; Metabolomics ; Multiple acyl-CoA dehydrogenase deficiency ; Seasonal pasture myopathy

刊名：The Veterinary Journal

出版年：2016

6.Fasting induces prominent proteomic changes in liver in very long chain Acyl-CoA dehydrogenase deficient mice

作者：Wei Wang^a ; ^b ; ^{wangwei@shchildren.com.cn" class="auth_mail" title="E-mail the corresponding author} ; Johan Palmfeldt^c ; Al-Walid Mohsen^d ; Niels Gregersen^c ; Jerry Vockley^b ; ^d

关键词：VLCAD deficiency ; β-oxidation ; Oxidative phosphorylation ; Proteomics ; HSP60 ; HSP610

刊名：Biochemistry and Biophysics Reports

出版年：2016

7.Lipolysis and lipophagy in lipid storage myopathies

作者：Corrado Angelini^a ; ^{corrado.angelini@unipd.it" class="auth_mail" title="E-mail the corresponding author} ; Anna Chiara Nascimbeni^b ; Giovanna Cenacchi^c ; Elisabetta Tasca^a

关键词：ATGL ; adipose triglyceride lipase ; ATP-ase ; adenosine tri-phosphatase ; CD ; carnitine deficiency ; CK ; creatine kinase ; COX ; cytochrome oxidase ; CPT ; carnitine-palmitoyl-transferase ; DAPI ; 4&prime ; 6-diamidin-2-phenylindole ; EMG ; electromyography ; ETF ; electron transfer flavoprotein ; FOXO ; fork head box protein ; GAPDH ; glyceraldehyde 3-phosphate dehydrogenase ; HSL ; hormone-sensitive lipase ; LC3 ; microtubule-associated proteins light chain-3 ; LSM ; lipid storage myopathy ; MADD ; multiple acyl-CoA deh

刊名：Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease

出版年：2016

8.G.P.57 - Exploration of muscle metabolism during exercise and muscle imaging in late-onset form of multiple acyl-coA dehydrogenase deficiency (MADD)

作者：S. Souvannanorath^* ; ¹ ; S. Hatem² ; M. Becquemin³ ; N. Zraik³ ; R. Bittar⁴ ; O. Rigal⁵ ; R. Carlier⁶ ; P. Carlier⁷ ; P. Laforê ; t¹

刊名：Neuromuscular Disorders

出版年：2015

9.G.P.58 - Not motoneuron disease - a case of late-onset multiple acyl-CoA dehydrogenase deficiency

作者：C. Merkel¹ ; N. Boy² ; K. Kappes-Horn¹ ; J. Reimann^* ; ¹

刊名：Neuromuscular Disorders

出版年：2015

10.Adult-onset multiple acyl CoA dehydrogenation deficiency associated with an abnormal isoenzyme pattern of serum lactate dehydrogenase

作者：Fuminobu Sugai^a ; ^{sugai1441@otemae.gr.jp} ; Kousuke Baba^a ; Keiko Toyooka^b ; Wen-Chen Liang^c ; Ichizo Nishino^c ; Misaki Yamadera^d ; Hisae Sumi^d ; Harutoshi Fujimura^b ; Yoshiro Nishikawa^a

关键词：Multiple acyl CoA dehydrogenation deficiency ; Electron transfer flavoprotein dehydrogenase ; Riboflavin ; Lactate dehydrogenase ; Isoenzyme

刊名：Neuromuscular Disorders

出版年：2012

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