搜索页_Fatty+acid+oxidation+defect

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1.Fatty Acid Oxidation is Impaired in An Orthologous Mouse Model of Autosomal Dominant Polycystic Kidney Disease

作者：Luis F. Menezes ; Cheng-Chao Lin ; Fang Zhou ; Gregory G. Germino

关键词：Polycystic kidney disease ; Autosomal Dominant Polycystic Kidney Disease ; Pkd1 ; ADPKD ; Mouse model ; Systems biology ; Metabolism ; Fatty acid oxidation ; Fatty acid oxidation defect ; Lipid metabolism ; Diet

刊名：EBioMedicine

出版年：2016

2.Defects in muscle branched-chain amino acid oxidation contribute to impaired lipid metabolism

作者：Carles Lerin¹ ; ² ; ³ ; ^{clerin@fsjd.org" class="auth_mail" title="E-mail the corresponding author} ; Allison B. Goldfine¹ ; ² ; Tanner Boes¹ ; Manway Liu⁴ ; Simon Kasif⁴ ; Jonathan M. Dreyfuss¹ ; ⁴ ; Ana Luisa De Sousa-Coelho¹ ; ² ; Grace Daher¹ ; Irini Manoli⁵ ; Justin R. Sysol⁵ ; Elvira Isganaitis¹ ; ² ; Niels Jessen¹ ; Laurie J. Goodyear¹ ; Kirk Beebe⁶ ; Walt Gall⁶ ; ⁷ ; Charles P. Venditti⁵ ; Mary-Elizabeth Patti¹ ; ² ; ^{mary.elizabeth.patti@joslin.harvard.edu" class="auth_mail" title="E-mail the corresponding author}

关键词：Insulin sensitivity ; BCAA ; Fatty acid oxidation ; TCA cycle

刊名：Molecular Metabolism

出版年：2016

3.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

4.Loss of Adipose Fatty Acid Oxidation Does Not Potentiate Obesity at Thermoneutrality

作者：Jieun Lee¹ ; ⁴ ; Joseph Choi¹ ; ⁴ ; Susan Aja² ; ⁴ ; Susanna Scafidi³ ; Michael J. Wolfgang¹ ; ⁴ ; ^{mwolfga1@jhmi.edu" class="auth_mail" title="E-mail the corresponding author}

刊名：Cell Reports

出版年：2016

5.Inborn Errors of Long-Chain Fatty Acid β-Oxidation Link Neural Stem Cell Self-Renewal to Autism

作者：Zhigang Xie¹ ; ^{zxie@tamhsc.edu" class="auth_mail" title="E-mail the corresponding author} ; Albert Jones² ; Jude T. Deeney² ; Seong Kwon Hur¹ ; Vytas A. Bankaitis¹

刊名：Cell Reports

出版年：2016

6.Reversal of Defective Mitochondrial Biogenesis in Limb-Girdle Muscular Dystrophy 2D by Independent Modulation of Histone and PGC-1α Acetylation

作者：Sarah Pambianco¹ ; ⁹ ; Matteo Giovarelli¹ ; ⁹ ; Cristiana Perrotta¹ ; Silvia Zecchini² ; Davide Cervia¹ ; ³ ; Ilaria Di Renzo¹ ; Claudia Moscheni¹ ; Michela Ripolone⁴ ; Raffaella Violano⁴ ; Maurizio Moggio⁴ ; Maria Teresa Bassi⁵ ; Pier Lorenzo Puri⁶ ; ⁷ ; Lucia Latella⁶ ; ⁸ ; Emilio Clementi² ; ⁵ ; ^{emilio.clementi@unimi.it} ; Clara De Palma² ; ¹⁰ ; ^{clara.depalma@unimi.it}

关键词：muscular dystrophy ; mitochondrial biogenesis ; histone acetylation ; fatty acid oxidation

刊名：Cell 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.Exercise training in metabolic myopathies

作者：J. Vissing ^{vissing@rh.dk" class="auth_mail" title="E-mail the corresponding author}

关键词：Metabolic myopathies ; Glycogenosis ; Exercise training ; Substrate supplementation ; Myoglobinuria

刊名：Revue Neurologique

出版年：2016

9.Impaired adipose tissue lipid storage, but not altered lipolysis, contributes to elevated levels of NEFA in type 2 diabetes. Degree of hyperglycemia and adiposity are important factors

作者：Maria J Pereira^a ; Stanko Skrtic^b ; ^c ; Petros Katsogiannos^a ; Niclas Abrahamsson^a ; Cherno O Sidibeh^a ; Santosh Dahgam^b ; Marianne Må ; nsson^b ; Ulf Risé ; rus^d ; Joel Kullberg^e ; Jan W Eriksson^a ; ^{jan.eriksson@medsci.uu.se" class="auth_mail" title="E-mail the corresponding author}

关键词：Lipolysis ; Type 2 diabetes ; Adipose tissue ; Metabolism ; Lipid storage

刊名：Metabolism

出版年：2016

10.Regulation of mitochondrial fatty acid 尾-oxidation in human: What聽can we learn from inborn fatty acid 尾-oxidation deficiencies?

作者：Jean Bastin

关键词：Mitochondrial fatty acid 尾-oxidation ; Inborn enzyme defects ; Newborn screening ; Pharmacological therapy ; PPAR ; Fibrates

刊名：Biochimie

出版年：January, 2014

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