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Elsevier电子期刊(30)

在“Elsevier电子期刊”中，命中：30条，耗时：小于0.01 秒

在所有数据库中总计命中：46条

1.Altered interaction and distribution of glycosaminoglycans and growth factors in mucopolysaccharidosis type I bone disease

关键词：Mucopolysaccharidosis ; Glycosaminoglycans ; Growth factors ; Bone disease ; Fibroblast growth factor ; Indian hedgehog

刊名：Bone

出版年：2016

2.A role for the human peroxisomal half-transporter ABCD3 in the oxidation of dicarboxylic acids

作者：Carlo W.T. van Roermund ; ^{c.vanroermund@amc.uva.nl" class="auth_mail} ; Lodewijk IJlst ; Tom Wagemans ; Ronald J.A. Wanders ; Hans R. Waterham

关键词：Peroxisomes ; Fatty acid oxidation ; ABC transporter ; Fatty acid transport ; ABCD3 ; Dicarboxylic acid

刊名：Biochimica et Biophysica Acta (BBA)/Molecular and Cell Biology of Lipids

出版年：April, 2014

3.Early phenotype prediction in mucopolysaccharidosis type I based on genotype, residual enzyme activity and clinical characteristics: a prerequisite for introduction of newborn screening

作者：Frits A. Wijburg ; S ; ra Kingma ; Eveline Langereis ; Clasine De Klerk ; Lida Zoetekouw ; Tom Wagemans ; Lodewijk IJlst ; Ronald J. W ; ers ; Naomi van Vlies

刊名：Molecular Genetics and Metabolism

出版年：February, 2014

4.Genistein and MPS I: opposing effects on GAG synthesis and storage in different cell models

作者：S ; ra D.K. Kingma ; Tom Wagemans ; Lodewijk IJlst ; Ronald J.A. W ; ers ; Frits A. Wijburg ; Naomi van Vlies

刊名：Molecular Genetics and Metabolism

出版年：February, 2014

5.Peroxisomes contribute to the acylcarnitine production when the carnitine shuttle is deficient

作者：Sara Violante ; Lodewijk IJlst ; Heleen te Brinke ; Janet Koster ; Isabel Tavares de Almeida ; Ronald J.A. W ; ers ; F¨¢tima V. Ventura ; S ; er M. Houten

关键词：C120 ; lauric acid ; CACT ; carnitine/acylcarnitine translocase ; CPT1 ; carnitine palmitoyltranferase 1 ; CPT2 ; carnitine palmitoyltransferase 2 ; CrAT ; carnitine acetyltranferase ; CrOT ; carnitine octanoyltransferase ; l-AC ; l-aminocarnitine ; LCFA ; long-chain f

刊名：Biochimica et Biophysica Acta (BBA)/Molecular and Cell Biology of Lipids

出版年：2013

6.Residual enzyme activity determines phenotypic severity in mucopolysaccharidosis type I

作者：Sandra Kingma ; Tom Wagemans ; Lodewijk IJlst ; Eveline Langereis ; Ronald Wanders ; Frits Wijburg ; Naomi van Vlies

刊名：Molecular Genetics and Metabolism

出版年：2013

7.New insights on the mechanisms of valproate-induced hyperammonemia: Inhibition of hepatic N-acetylglutamate synthase activity by valproyl-CoA

作者：Cá ; tia C.P. Aires¹ ; ² ; Arno van Cruchten¹ ; Lodewijk IJlst¹ ; Isabel Tavares de Almeida² ; Marinus Duran¹ ; Ronald J.A. Wanders¹ ; ^† ; ; Margarida F.B. Silva² ; ^† ; ; ^{mbsilva@ff.ul.pt"" rel=""nofollow}

关键词：Hyperammonemia ; Urea cycle ; Drug-induced liver injury ; Drug-induced hepatotoxicity ; N-acetylglutamate synthase ; N-acetylglutamate ; Valproate ; Valproyl-CoA

刊名：Journal of Hepatology

出版年：2011

8.Inhibition of hepatic carnitine palmitoyl-transferase I (CPT IA) by valproyl-CoA as a possible mechanism of valproate-induced steatosis

作者：Cá ; tia C.P. Aires ; Lodewijk IJlst ; Femke Stet ; Carina Prip-Buus ; Isabel Tavares de Almeida ; Marinus Duran ; Ronald J.A. W ; ers ; Margarida F.B. Silva

关键词：Carnitine palmitoyl-transferase I ; Valproic acid ; Malonyl-CoA ; Fatty acid oxidation ; Drug-induced hepatic steatosis

刊名：Biochemical Pharmacology

出版年：2010

9.Carnitine palmitoyltransferase 2: New insights on the substrate specificity and implications for acylcarnitine profiling

作者：Sara Violante ; Lodewijk IJlst ; Henk van Lenthe ; Isabel Tavares de Almeida ; Ronald J. W ; ers ; Fá ; tima V. Ventura

关键词：Acylcarnitine ; Carnitine palmitoyltransferase 2 (CPT2) ; Long-chain acyl-CoA ; Fatty acid oxidation (FAO) ; Mitochondrial Trifunctional Protein (MTP)

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

出版年：2010

10.Characterization of l-aminocarnitine, an inhibitor of fatty acid oxidation

作者：Malika Chegary ; Heleen te Brinke ; Mirjam Doolaard ; Lodewijk IJlst ; Frits A. Wijburg ; Ronald J.A. W ; ers ; S ; er M. Houten

关键词：l-Aminocarnitine ; Peroxisome proliferator-activated receptors ; Carnitine palmitoyltransferase ; Inborn errors of metabolism ; Fatty acid β ; -oxidation

刊名：Molecular Genetics and Metabolism

出版年：2008

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