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在“Elsevier电子期刊”中，命中：406条，耗时：0.1279389 秒

在所有数据库中总计命中：628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.The molecular structure of thio-ether fatty acids influences PPAR-dependent regulation of lipid metabolism

作者：Jenny Lund^a ; ^&dagger ; ; Camilla Stensrud^a ; ^&dagger ; ; Rajender^b ; ^c ; Pavol Bohov^d ; G. Hege Thoresen^a ; ^e ; Rolf K. Berge^d ; ^f ; Michael Wright^b ; ^g ; Ahmed Kamal^c ; Arild C. Rustan^a ; Andrew D. Miller^c ; ^g ; ^h ; Jon Skorve^d ; ^{jon.skorve@k2.uib.no" class="auth_mail" title="E-mail the corresponding author}

关键词：THEFA ; thio-ether fatty acid ; FA ; fatty acid ; MUFA ; monounsaturated fatty acid ; SFA ; saturated fatty acid ; TTA ; 2-(tetradecylthio)acetic acid ; TTA-PC ; 1 ; 2-di-[2-(tetradecylthio)acetyl]-sn-glycero-3-phosphocholine ; TTA-MAG ; 1-[2-(tetradecylthio)acetyl]-sn-glycerol ; dTTA ; 2-(tetradec-[7-enyl]thio)acetic acid ; dTTA-PC ; 1 ; 2-di-[2-(tetradec-[7-enyl]thio)acetyl]-sn-glycero-3-phosphocholine ; lyso-dTTA-PC ; 1-[2-(tetradec-[7-enyl]thio)acetyl]-sn-glycero-3-phosphocholine ; dTTA-MAG ; 1-[2-(tetradec-[7-enyl]

刊名：Bioorganic & Medicinal Chemistry

出版年：2016

3.Triheptanoin treatment in patients with pediatric cardiomyopathy associated with long chain-fatty acid oxidation disorders

作者：J. Vockley^a ; ^{vockleyg@upmc.edu" class="auth_mail" title="E-mail the corresponding author} ; J. Charrow^b ; J. Ganesh^c ; M. Eswara^d ; G.A. Diaz^e ; E. McCracken^a ; R. Conway^f ; G.M. Enns^g ; J. Starr^h ; R. Wang^h ; J.E. Abdenur^h ; J. Sanchez-de-Toledo^a ; D.L. Marsdenⁱ

关键词：Cardiomyopathy ; Metabolic disorders ; Fatty acid oxidation disorders ; Triheptanoin

刊名：Molecular Genetics and Metabolism

出版年：2016

4.A case of sudden unexpected infant death involving a homozygotic twin with the thermolabile CPT2 variant, accompanied by rotavirus infection and treatment with an antibiotic containing pivalic acid

作者：Yoichiro Takahashi^a ; ^{y.takahashi@gunma-u.ac.jp" class="auth_mail" title="E-mail the corresponding author} ; Rie Sano^a ; Yoshihiko Kominato^a ; Rieko Kubo^a ; Keiko Takahashi^a ; Tamiko Nakajima^a ; Haruo Takeshita^b ; Takashi Ishige^c

关键词：Sudden unexpected infant death ; Fatty acid oxidation ; CPT2 ; Pivalic acid

刊名：Legal Medicine

出版年：2016

5.CYP4F2 affects phenotypic outcome in adrenoleukodystrophy by modulating the clearance of very long-chain fatty acids

作者：Catherine E. van Engen^a ; Rob Ofman^a ; Inge M.E. Dijkstra^a ; Tessa Jacobs van Goethem^a ; Eveline Verheij^a ; Jennifer Varin^b ; Michel Vidaud^b ; Ronald J.A. Wanders^a ; Patrick Aubourg^c ; ^d ; ¹ ; Stephan Kemp^a ; ¹ ; ^{s.kemp@amc.uva.nl" class="auth_mail" title="E-mail the corresponding author} ; Mathieu Barbier^c ; ¹ ; ²

关键词：Adrenoleukodystrophy ; Modifier gene ; Dicarboxylic acids ; Cytochrome P450 ; Omega-oxidation ; Peroxisomes

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

出版年：2016

6.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

7.Carnitine palmitoyltransferase 1C: From cognition to cancer

作者：Nú ; ria Casals^a ; ^b ; ^{ncasals@uic.es" class="auth_mail" title="E-mail the corresponding author} ; Victor Zammit^c ; ^{V.A.Zammit@warwick.ac.uk" class="auth_mail" title="E-mail the corresponding author} ; Laura Herrero^a ; ^d ; Rut Fadó ; ^a ; ^b ; Rosalí ; a Rodrí ; guez-Rodrí ; guez^a ; ^b ; Dolors Serra^a ; ^d

关键词：ACC ; acetyl-CoA carboxylase ; ACO ; aconitase ; AICAR ; 5-aminoimidazole-4-1-b-d-ribofuranoside ; AMPK ; AMP-dependent protein kinase ; Arc ; Arcuate ; BSX ; brain-specific homeobox ; cAMP ; cyclic AMP ; CNS ; central nervous system ; COT ; carnitine octanoyltransferase ; CPT ; carnitine palmitoyltransferase ; CrAT ; carnitine acetyltransferase ; CREB ; cAMP response element-binding protein ; DG ; deoxyglucose ; ER ; endoplasmic reticulum ; ERRα ; estrogen-related receptor α ; FA ; fatty acid ; FAO ; fatty acid oxidation ; FAS

刊名：Progress in Lipid Research

出版年：2016

8.Long-term major clinical outcomes in patients with long chain fatty acid oxidation disorders before and after transition to triheptanoin treatment鈥擜 retrospective chart review

作者：Jerry Vockley^a ; ^b ; ^{gerard.vockley@chp.edu" class="auth_mail" title="E-mail the corresponding author} ; Deborah Marsden^c ; Elizabeth McCracken^a ; Stephanie DeWard^a ; Amanda Barone^a ; Kristen Hsu^c ; Emil Kakkis^c

关键词：CACT ; carnitine acylcarnitine translocase ; CM ; cardiomyopathy ; CRF ; case report forms ; ECHO ; echocardiography ; ER ; emergency room ; LC-FAOD ; long chain fatty acid oxidation disorder ; MCT ; Medium Chain Triglyceride ; TFP ; trifunctional protein ; CK ; creatine kinase ; CPT ; carnitine palmitoyltransferase deficiencies ; ICU ; intensive care unit ; IQR ; interquartile range ; IV ; intravenous ; LCHAD ; long-chain 3-hydroxy-acyl-CoA dehydrogenase ; VLCAD ; very long chain acyl-CoA dehydrogenase

刊名：Molecular Genetics and Metabolism

出版年：2015

9.Peripheral neuropathy in patients with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency - A follow-up EMG study of 12 patients

作者：Immonen Tuuli^a ; ^{tuuli.immonen@hus.fi" class="auth_mail" title="E-mail the corresponding author} ; Ahola Emilia^a ; Toppila Jussi^b ; Lapatto Risto^a ; Tyni Tiina^a ; Lauronen Leena^b

关键词：Fatty acid oxidation disorders ; Inborn errors of metabolism ; Mitochondrial fatty acid β-oxidation ; Polyneuropathy

刊名：European Journal of Paediatric Neurology

出版年：2016

10.Altered heart proteome in fructose-fed Fisher 344 rats exposed to bisphenol A

作者：S.A. Ljunggren^a ; ^{Stefan.ljunggren@liu.se" class="auth_mail" title="E-mail the corresponding author} ; M. Iggland^b ; ^{Madeleine.iggland@liu.se" class="auth_mail" title="E-mail the corresponding author} ; M. Rö ; nn^c ; ^{Monika.ronn@medsci.uu.se" class="auth_mail" title="E-mail the corresponding author} ; L. Lind^d ; ^{Lars.lind@medsci.uu.se" class="auth_mail" title="E-mail the corresponding author} ; P.M. Lind^c ; ^{Monica.lind@medsci.uu.se" class="auth_mail" title="E-mail the corresponding author} ; H. Karlsson^a ; ^{helen.m.karlsson@liu.se" class="auth_mail" title="E-mail the corresponding author}

关键词：2DE ; two-dimensional gel electrophoresis ; BPA ; Bisphenol A ; FA ; Formic acid ; FDR ; False discovery rate ; DHB ; 2 ; 5-dihydroxybenzoic acid ; MALDI-TOF ; matrix associated laser desorption/ionization time-of-flight ; MS ; mass spectrometry ; MS/MS ; tandem mass spectrometry ; nLC ; nano-liquid chromatography ; S1P ; sphingosine-1-phosphate ; TFA ; Trifluoroacetic acid

刊名：Toxicology

出版年：2016

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