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Elsevier电子期刊(90)

在“Elsevier电子期刊”中，命中：90条，耗时：0.0569681 秒

在所有数据库中总计命中：118条

1.“Role of peroxisomes in human lipid metabolism and its importance for neurological development”

作者：Ronald J.A. Wanders^a ; ; Bwee Tien Poll-The^b ; ^{r.j.wanders@amc.uva.nl}

关键词：Peroxisomes ; Peroxisomal disorders ; Lipidomics ; Lipids ; Fatty acids ; Mitochondria

刊名：Neuroscience Letters

出版年：2017

2.A new multiplex method for the diagnosis of peroxisomal disorders allowing simultaneous determination of plasma very-long-chain fatty acids, phytanic, pristanic, docosahexaenoic and bile acids by high-performance liquid chromatography-atmospheric pressure chemical ionization-tandem mass spectrometry

作者：Michela Semeraro^a ; ¹ ; ^{michela.semeraro@opbg.net" class="auth_mail" title="E-mail the corresponding author} ; Cristiano Rizzo^a ; ¹ ; Sara Boenzi^a ; Marco Cappa^b ; Enrico Bertini^c ; Giacomo Antonetti^a ; Carlo Dionisi-Vici^a

关键词：PDs ; peroxisomal disorders ; LC-MS/MS ; liquid chromatography-tandem mass spectrometry ; CV ; coefficient of variation ; VLCFAs ; very-long-chain fatty acids ; Pri ; pristanic acid ; PUFAs ; polyunsaturated fatty acids ; DHA ; docosahexaenoic acid ; Phy ; phytanic acid ; PBDs ; peroxisomal biogenesis disorders ; GC&ndash ; MS ; gas chromatography&ndash ; mass spectrometry ; ESI-MS/MS ; electrospray ionization tandem mass spectrometry ; UPLC ; ultra-performance liquid chromatography ; DAABD-AE ; 4-[2-(N ; N-dimethylamino) eth

刊名：Clinica Chimica Acta

出版年：2016

3.Diagnosis of a mild peroxisomal phenotype with next-generation sequencing

作者：Meredith J. Ventura^a ; Dianna Wheaton^b ; ^c ; Mingchu Xu^d ; David Birch^b ; ^c ; Sara J. Bowne^e ; Lori S. Sullivan^e ; Stephen P. Daiger^e ; Annette E. Whitney^f ; Richard O. Jones^g ; Ann B. Moser^g ; Rui Chen^d ; Michael F. Wangler^d ; ^h ; ^{michael.wangler@bcm.edu}

关键词：PEX1 p.G843D ; Zellweger syndrome spectrum ; Peroxisomal biogenesis disorders ; Usher syndrome

刊名：Molecular Genetics and Metabolism Reports

出版年：2016

4.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

5.Low bone mineral density is a common feature of Zellweger spectrum disorders

作者：Eric T. Rush^a ; ^b ; ^c ; ^{erush@unmc.edu" class="auth_mail" title="E-mail the corresponding author} ; Jennifer L. Goodwin^a ; Nancy E. Braverman^d ; William B. Rizzo^a ; ^c

关键词：Peroxisomal biogenesis disorder ; Zellweger spectrum disorder ; Bone mineral density ; Bone densitometry ; PPAR-gamma ; Pathologic fracture ; Bisphosphonate

刊名：Molecular Genetics and Metabolism

出版年：2016

6.Multiple paths to peroxisomes: Mechanism of peroxisome maintenance in mammals

作者：Rong Hua ; Peter K. Kim ; ^{pkim@sickkids.ca" class="auth_mail" title="E-mail the corresponding author}

关键词：CHO ; Chinese hamster ovary ; DLP1 ; dynamin-like protein 1 ; ER ; endoplasmic reticulum ; FIS1 ; fission 1 ; H. polymorpha ; Hansenula polymorpha ; MFF ; mitochondrial fission factor ; MAPL ; mitochondria-anchored protein ligase ; MDV ; mitochondria-derived vesicle ; mPTS ; integral membrane targeting sequence ; PAGFP ; photo-activable GFP ; PBD ; peroxisomal biogenesis disorders ; PMP ; peroxisomal membrane protein ; PPAR ; peroxisome-proliferator-activated receptors ; PTS ; peroxisomal targeting signals ; S. cerevisiae

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

出版年：2016

7.Pioglitazone significantly prevented decreased rate of neural differentiation of mouse embryonic stem cells which was reduced by Pex11β knock-down

作者：M. Esmaeili^a ; K. Ghaedi^a ; ^b ; ^{kamranghaedi@royaninstitute.org" class="auth_mail" title="E-mail the corresponding author} ; A. Shoaraye Nejati^a ; M. Nematollahi^a ; H. Shiralyian^a ; M.H. Nasr-Esfahani^a ; ^{mh_nasr@royaninstitute.org" class="auth_mail" title="E-mail the corresponding author}

关键词：Acox1 ; acyl-coenzyme A oxidase 1 ; Agps ; alkylglycerole phosphate synthase ; BSA ; bovine serum albumin ; DMSO ; dimethyl-sulfoxide ; Dox ; Doxycycline ; EBs ; embryoid bodies ; ES-FCS ; embryonic stem cells qualified-FCS ; FCS ; fetal calf serum ; GAPDH ; glyceraldehyde 3-phosphate dehydrogenase ; mESC ; mouse embryonic stem cell ; NPCs ; Neural progenitor cells ; Pax6 ; paired box gene 6 ; PBDs ; peroxisomal biogenesis disorders ; PBFE ; peroxisomal bi-functional enzyme ; PBS ; phosphate-buffered saline ; Pio ; pioglitazo

刊名：Neuroscience

出版年：2016

8.Simultaneous quantitation of hexacosanoyl lysophosphatidylcholine, amino acids, acylcarnitines, and succinylacetone during FIA-ESI-MS/MS analysis of dried blood spot extracts for newborn screening

作者：Christopher A. Haynes ; ^{cph7@cdc.gov" class="auth_mail" title="E-mail the corresponding author} ; Ví ; ctor R. De Jesús

关键词：Flow-injection analysis ; Tandem mass spectrometry ; Newborn screening ; Dried blood spot ; X-linked adrenoleukodystrophy

刊名：Clinical Biochemistry

出版年：2016

9.Simultaneous quantitation of hexacosanoyl lysophosphatidylcholine, amino acids, acylcarnitines, and succinylacetone during FIA-ESI-MS/MS analysis of dried blood spot extracts for newborn screening

作者：Christopher A. Haynes ; ^{cph7@cdc.gov" class="auth_mail" title="E-mail the corresponding author} ; Ví ; ctor R. De Jesús

关键词：Flow-injection analysis ; Tandem mass spectrometry ; Newborn screening ; Dried blood spot ; X-linked adrenoleukodystrophy

刊名：Clinical Biochemistry

出版年：2016

10.Molecular basis of peroxisomal biogenesis disorders caused by defects in peroxisomal matrix protein import

作者：Shirisha Nagotu ; Vishal C. Kalel ; Ralf Erdmann ; Harald W. Platta

关键词：Peroxisome biogenesis disorders ; Zellweger syndrome spectrum ; PEX ; Peroxin ; Ubiquitination

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

出版年：2012

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