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在“Elsevier电子期刊”中，命中：6条，耗时：0.0540102 秒

在所有数据库中总计命中：4条

1.Hepatic dysfunction in peroxisomal disorders

作者：Myriam Baes^a ; ^{myriam.baes@pharm.kuleuven.be" class="auth_mail" title="E-mail the corresponding author} ; Paul P. Van Veldhoven^b ; ^{paul.vanveldhoven@med.kuleuven.be" class="auth_mail" title="E-mail the corresponding author}

关键词：ABC ; ATP-binding cassette ; ACAA1 ; acetyl-CoA acyltransferase ; ACOX ; acyl-CoA oxidase ; BAAT ; bile acid CoA-amino acid N-acyl transferase ; C24-bile acid ; cholanoic acid ; C27-bile acid ; cholestanoic acid ; CDCA ; chenodeoxycholic acid ; Cn-DCA ; straight chain dicarboxylic acid with n C atoms ; DCA ; dicarboxylic acid ; DHA ; docosahexaenoic acid ; DHCA ; dihydroxycholestanoic acid ; HAO ; 2-hydroxy acid oxidase ; ER ; endoplasmic reticulum ; MFP ; multifunctional protein ; PBD ; peroxisome biogenesis disorder ; PMP

刊名：Biochimica et Biophysica Acta (BBA)-Molecular Cell Research

出版年：2016

2.Peroxisome biogenesis disorders in the Zellweger spectrum: An overview of current diagnosis, clinical manifestations, and treatment guidelines

关键词：AAV ; adeno-associated virus ; AED ; anti-epileptic drug(s) ; DHA ; docosahexaenoic acid ; DHCA ; dihydroxycholestanoic acid ; ERG ; electroretinogram ; G-tube ; gastrostomy tube ; IRD ; infantile Refsum disease ; LC-MS/MS ; liquid chromatography/tandem mass spectrometry ; NALD ; neonatal adrenoleukodystrophy ; OCT ; optical coherence tomography ; PBD ; peroxisome biogenesis disorder ; PBD-ZSD ; peroxisome biogenesis disorder-Zellweger spectrum disorder ; THCA ; trihydroxycholestanoic acid ; VLCFA ; very long chain fatty

刊名：Molecular Genetics and Metabolism

出版年：2016

3.The Pex1-G844D mouse: A model for mild human Zellweger spectrum disorder

作者：Shandi Hiebler^a ; ¹ ; Tomohiro Masuda^b ; ¹ ; Joseph G. Hacia^c ; Ann B. Moser^a ; ^d ; Phyllis L. Faust^e ; Anita Liu^a ; Nivedita Chowdhury^a ; Ning Huang^c ; Amanda Lauer^f ; Jean Bennett^g ; Paul A. Watkins^a ; ^d ; Donald J. Zack^b ; ^h ; ⁱ ; ^j ; ^k ; Nancy E. Braverman^l ; ^m ; Gerald V. Raymond^a ; ^d ; ² ; Steven J. Steinberg^a ; ^d ; ^h ; ^{ssteinb8@jhmi.edu" class="auth_mail}

关键词：ABR ; auditory brainstem response ; C26 ; 0-LPC ; C26 ; 0-lysophosphatidylethanolamine ; DHCA ; dihydroxycholestanoic acid ; DMSO ; dimethylsulfoxide ; ERG ; electroretinogram ; FBS-EMEM ; fetal bovine serum-Eagle's minimum essential media ; h ; hour ; IRD ; infantile Refsum disease ; LC&ndash ; MS/MS ; liquid chromatography tandem mass spectrometry ; NALD ; neonatal adrenoleukodystrophy ; PE ; phosphatidylethanolamine ; PEX ; peroxins ; PEX1-G843D ; human PEX1 mutation c.2528G> ; A (p. G843D) ; Pex1-G844D ; murine Pex1 mutatio

刊名：Molecular Genetics and Metabolism

出版年：April, 2014

4.Peroxisomal ABC transporters: Structure, function and role in disease

作者：Masashi Morita ; Tsuneo Imanaka

关键词：ABCD ; ATP-binding cassette protein subfamily D ; AMN ; adrenomyeloneuropathy ; CCALD ; child cerebral ALD ; CNS ; central nervous system ; DHCA ; dihydroxycholestanoic acid ; DHA ; docosahexaenoic acid ; GFP ; green fluorescent protein ; iPS ; induced pluripotent stem

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

出版年：2012

5.Toxicity of peroxisomal C₂₇-bile acid intermediates

作者：Sacha Ferdin ; usse ; Simone Denis ; Georges Dacremont ; Ronald J.A. W ; ers

关键词：Oxidative phosphorylation ; ROS production ; Cell viability ; Peroxisomal disorders ; Bile acid biosynthesis

刊名：Molecular Genetics and Metabolism

出版年：2009

6.Molecular basis of d-bifunctional protein deficiency

作者：Mö ; ller ; G. ; van Grunsven ; E.G. ; Wanders ; R.J.A. ; Adamski ; J.

关键词：d-Bifunctional protein ; MFP-2 ; 17β ; -HSD4 ; Peroxisomal disorders ; β ; -Oxidation ; Inherited disease ; Polymorphisms ; aa ; amino acid ; d-BP ; d-bifunctional protein ; DHCA ; dihydroxycholestanoic acid ; 17β ; -HSD ; 17β ; -hydroxysteroid dehydrogenase ; l

刊名：Molecular and Cellular Endocrinology

出版年：2001

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