Di- trihydroxycholestanoic acid oxidation/Bile acid synthesis by Pubmed references

YEAR FIRST AUTHOR JOURNAL TITLE
2009 Daniela Dietrich Mol. Biol. Cell Mutations in the Arabidopsis peroxisomal ABC transporter COMATOSE allow differentiation between multiple functions in planta: insights from an allelic series.[Pubmed]
2009 Liu Liu J. Lipid Res. ABCD2 is abundant in adipose tissue and opposes the accumulation of dietary erucic acid (C22:1) in fat.[Pubmed]
2009 Jaspreet Singh J. Lipid Res. Silencing of Abcd1 and Abcd2 genes sensitizes astrocytes for inflammation: implication for X-adrenoleukodystrophy.[Pubmed]
2009 Pedro Brites Brain Plasmalogens participate in very-long-chain fatty acid-induced pathology.[Pubmed]
2009 Stéphane Fourcade Am. J. Physiol. Endocrinol. Metab. A key role for the peroxisomal ABCD2 transporter in fatty acid homeostasis.[Pubmed]
2009 Yoshinori Kashiwayama Exp. Cell Res. 70-kDa peroxisomal membrane protein related protein (P70R/ABCD4) localizes to endoplasmic reticulum not peroxisomes, and NH2-terminal hydrophobic property determines the subcellular localization of ABC subfamily D proteins.[Pubmed]
2008 Rita Denti Neurochem. Int. RNAi-mediated silencing of ABCD3 gene expression in rat C6 glial cells: a model system to study PMP70 function.[Pubmed]
2008 Lodewijk Visser FASEB J. The human peroxisomal ABC half transporter ALDP functions as a homodimer and accepts acyl-CoA esters.[Pubmed]
2008 M Engelen Biochim. Biophys. Acta Cholesterol-deprivation increases mono-unsaturated very long-chain fatty acids in skin fibroblasts from patients with X-linked adrenoleukodystrophy.[Pubmed]
2008 Stéphane Fourcade Hum. Mol. Genet. Early oxidative damage underlying neurodegeneration in X-adrenoleukodystrophy.[Pubmed]
2008 M Morita J. Inherit. Metab. Dis. Baicalein 5,6,7-trimethyl ether activates peroxisomal but not mitochondrial fatty acid beta-oxidation.[Pubmed]
2007 Norimasa Takahashi J. Neurochem. Adrenoleukodystrophy: subcellular localization and degradation of adrenoleukodystrophy protein (ALDP/ABCD1) with naturally occurring missense mutations.[Pubmed]
2007 Sally Hooks Biochem. J. The Arabidopsis ALDP protein homologue COMATOSE is instrumental in peroxisomal acetate metabolism.[Pubmed]
2007 F Thevenieau Fungal Genet. Biol. Characterization of Yarrowia lipolytica mutants affected in hydrophobic substrate utilization.[Pubmed]
2007 Rea Rea Plant ATP-binding cassette transporters.[Pubmed]
2007 Lodewijk Visser Biochem. J. Metabolite transport across the peroxisomal membrane.[Pubmed]
2007 Hossein Burke Transl Res Mitochondrial ATP-binding cassette proteins.[Pubmed]
2007 Stephan Wanders Pflugers Arch. The peroxisomal ABC transporter family.[Pubmed]
2007 Wierzbicki Wierzbicki Biochem. Soc. Trans. Peroxisomal disorders affecting phytanic acid alpha-oxidation: a review.[Pubmed]
2007 Jyh-Feng Lu Lab. Invest. The role of peroxisomal ABC transporters in the mouse adrenal gland: the loss of Abcd2 (ALDR), Not Abcd1 (ALD), causes oxidative damage.[Pubmed]
2007 Stephan Kemp Mol. Genet. Metab. X-linked adrenoleukodystrophy: very long-chain fatty acid metabolism, ABC half-transporters and the complicated route to treatment.[Pubmed]
2007 Anca Nenicu Biol. Reprod. Peroxisomes in human and mouse testis: differential expression of peroxisomal proteins in germ cells and distinct somatic cell types of the testis.[Pubmed]
2007 Stephen Styles J. Lipid Res. Quantification and regulation of the subcellular distribution of bile acid coenzyme A:amino acid N-acyltransferase activity in rat liver.[Pubmed]
2007 Antonella Pellicoro Hepatology Human and rat bile acid-CoA:amino acid N-acyltransferase are liver-specific peroxisomal enzymes: implications for intracellular bile salt transport.[Pubmed]
2007 Yoshinori Kashiwayama J. Biol. Chem. Hydrophobic regions adjacent to transmembrane domains 1 and 5 are important for the targeting of the 70-kDa peroxisomal membrane protein.[Pubmed]
2006 Masashi Morita Biol. Pharm. Bull. ATP-binding and -hydrolysis activities of ALDP (ABCD1) and ALDRP (ABCD2), human peroxisomal ABC proteins, overexpressed in Sf21 cells.[Pubmed]
2006 Fabien Gueugnon Biochem. Biophys. Res. Commun. A novel cell model to study the function of the adrenoleukodystrophy-related protein.[Pubmed]
2006 Johannes Berger Biochim. Biophys. Acta X-linked adrenoleukodystrophy: clinical, biochemical and pathogenetic aspects.[Pubmed]
2005 Catherine Gondcaille J. Cell Biol. Phenylbutyrate up-regulates the adrenoleukodystrophy-related gene as a nonclassical peroxisome proliferator.[Pubmed]
2005 Clara Guimarães J. Hum. Genet. Probing substrate-induced conformational alterations in adrenoleukodystrophy protein by proteolysis.[Pubmed]
2005 Helen Pinfield-Wells Plant J. Sucrose rescues seedling establishment but not germination of Arabidopsis mutants disrupted in peroxisomal fatty acid catabolism.[Pubmed]
2005 Sidney Mak Horm. Res. Novel insertion 496_497insG creating a stop codon D194X in a Chinese family with X-Linked adrenoleukodystrophy.[Pubmed]
2005 Shan Zha Prostate Peroxisomal branched chain fatty acid beta-oxidation pathway is upregulated in prostate cancer.[Pubmed]
2005 Yoshinori Kashiwayama Biochim. Biophys. Acta Role of Pex19p in the targeting of PMP70 to peroxisome.[Pubmed]
2005 K Dunster Clin. Exp. Immunol. Limkain b1, a novel human autoantigen localized to a subset of ABCD3 and PXF marked peroxisomes.[Pubmed]
2005 Muriel Asheuer Hum. Mol. Genet. Decreased expression of ABCD4 and BG1 genes early in the pathogenesis of X-linked adrenoleukodystrophy.[Pubmed]
2005 Isidre Ferrer Hum. Mol. Genet. Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage.[Pubmed]
2004 Mark IJlst J. Cell. Sci. The peroxisomal lumen in Saccharomyces cerevisiae is alkaline.[Pubmed]
2004 Martin Fulda Plant Cell Peroxisomal Acyl-CoA synthetase activity is essential for seedling development in Arabidopsis thaliana.[Pubmed]
2004 Charles Peters Blood Cerebral X-linked adrenoleukodystrophy: the international hematopoietic cell transplantation experience from 1982 to 1999.[Pubmed]
2004 Aurora Pujol Hum. Mol. Genet. Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy.[Pubmed]
2004 Kohichiroh Yasui Cancer Res. Alteration in copy numbers of genes as a mechanism for acquired drug resistance.[Pubmed]
2004 Kovacs Kovacs Mol. Cell. Biol. Disturbed cholesterol homeostasis in a peroxisome-deficient PEX2 knockout mouse model.[Pubmed]
2004 Pedro Guimarães Biochim. Biophys. Acta Mouse liver PMP70 and ALDP: homomeric interactions prevail in vivo.[Pubmed]
2004 Inderjit Singh Brain Res. Impaired peroxisomal function in the central nervous system with inflammatory disease of experimental autoimmune encephalomyelitis animals and protection by lovastatin treatment.[Pubmed]
2004 Hiroyuki Shibata J. Biol. Chem. Domain architecture and activity of human Pex19p, a chaperone-like protein for intracellular trafficking of peroxisomal membrane proteins.[Pubmed]
2003 Sonia Benhamida Mol. Ther. Transduced CD34+ cells from adrenoleukodystrophy patients with HIV-derived vector mediate long-term engraftment of NOD/SCID mice.[Pubmed]
2003 Mikinori Kurisu Biochem. Biophys. Res. Commun. Existence of catalase-less peroxisomes in Sf21 insect cells.[Pubmed]
2003 J-F McGuinness Mol. Cell. Biol. Role of ALDP (ABCD1) and mitochondria in X-linked adrenoleukodystrophy.[Pubmed]
2003 Pablo Landgraf Eur. J. Cell Biol. Targeting of the human adrenoleukodystrophy protein to the peroxisomal membrane by an internal region containing a highly conserved motif.[Pubmed]
2003 Stéphanie Boisnard Mol. Microbiol. Overexpression of a human and a fungal ABC transporter similarly suppresses the differentiation defects of a fungal peroxisomal mutant but introduces pleiotropic cellular effects.[Pubmed]
2003 Yun Shen-Ong Cancer Res. Expression profiling identifies a novel alpha-methylacyl-CoA racemase exon with fumarate hydratase homology.[Pubmed]
2003 Tracy Setchell Gastroenterology Liver disease caused by failure to racemize trihydroxycholestanoic acid: gene mutation and effect of bile acid therapy.[Pubmed]
2003 Isao Ilieva Neurol. Res. Age-related changes in peroxisomal membrane protein 70 and superoxide dismutase 1 in transgenic G93A mice.[Pubmed]
2003 Ben Geuze Mol. Biol. Cell Involvement of the endoplasmic reticulum in peroxisome formation.[Pubmed]
2003 Johannes Berger Adv. Exp. Med. Biol. Lessons from knockout mice II: Mouse models for peroxisomal disorders with single protein deficiency.[Pubmed]
2002 Nobuyuki Shimozawa Biochem. Biophys. Res. Commun. A novel aberrant splicing mutation of the PEX16 gene in two patients with Zellweger syndrome.[Pubmed]
2002 Noriyo Hashiguchi Mol. Biol. Cell Peroxisomes are formed from complex membrane structures in PEX6-deficient CHO cells upon genetic complementation.[Pubmed]
2002 Deyanira Corzo Am. J. Hum. Genet. Contiguous deletion of the X-linked adrenoleukodystrophy gene (ABCD1) and DXS1357E: a novel neonatal phenotype similar to peroxisomal biogenesis disorders.[Pubmed]
2002 Isabelle Weinhofer Hum. Mol. Genet. Cholesterol regulates ABCD2 expression: implications for the therapy of X-linked adrenoleukodystrophy.[Pubmed]
2002 Steven Footitt EMBO J. Control of germination and lipid mobilization by COMATOSE, the Arabidopsis homologue of human ALDP.[Pubmed]
2002 J Gärtner Endocr. Res. Functional characterization of the adrenoleukodystrophy protein (ALDP) and disease pathogenesis.[Pubmed]
2002 Makoto Hayashi Plant Cell Physiol. Ped3p is a peroxisomal ATP-binding cassette transporter that might supply substrates for fatty acid beta-oxidation.[Pubmed]
2002 Tanja Mayerhofer Biochem. Biophys. Res. Commun. Two splice variants of human PEX19 exhibit distinct functions in peroxisomal assembly.[Pubmed]
2002 S Ferdinandusse Am. J. Hum. Genet. Reinvestigation of peroxisomal 3-ketoacyl-CoA thiolase deficiency: identification of the true defect at the level of d-bifunctional protein.[Pubmed]
2002 Aurora Pujol Hum. Mol. Genet. Late onset neurological phenotype of the X-ALD gene inactivation in mice: a mouse model for adrenomyeloneuropathy.[Pubmed]
2002 S Ferdinandusse J. Lipid Res. Stereochemistry of the peroxisomal branched-chain fatty acid alpha- and beta-oxidation systems in patients suffering from different peroxisomal disorders.[Pubmed]
2002 Kouichi Tanaka J. Biol. Chem. ATP binding/hydrolysis by and phosphorylation of peroxisomal ATP-binding cassette proteins PMP70 (ABCD3) and adrenoleukodystrophy protein (ABCD1).[Pubmed]
2002 Aritoshi Iida J. Hum. Genet. Catalog of 605 single-nucleotide polymorphisms (SNPs) among 13 genes encoding human ATP-binding cassette transporters: ABCA4, ABCA7, ABCA8, ABCD1, ABCD3, ABCD4, ABCE1, ABCF1, ABCG1, ABCG2, ABCG4, ABCG5, and ABCG8.[Pubmed]
2002 Yoshinori Kashiwayama Biochem. Biophys. Res. Commun. Nucleotide-induced conformational changes of PMP70, an ATP binding cassette transporter on rat liver peroxisomal membranes.[Pubmed]
2001 T Voorn-Brouwer J. Cell. Sci. Peroxisomal membrane proteins are properly targeted to peroxisomes in the absence of COPI- and COPII-mediated vesicular transport.[Pubmed]
2001 W Kovacs Histochem. Cell Biol. Localization of mRNAs encoding peroxisomal proteins in cell culture by non-radioactive in situ hybridization. Comparison of rat and human hepatoma cells and their responses to two divergent hypolipidemic drugs.[Pubmed]
2001 A Gurvitz J. Biol. Chem. Saccharomyces cerevisiae Adr1p governs fatty acid beta-oxidation and peroxisome proliferation by regulating POX1 and PEX11.[Pubmed]
2001 A Gurvitz J. Biol. Chem. Peroxisomal degradation of trans-unsaturated fatty acids in the yeast Saccharomyces cerevisiae.[Pubmed]
2001 S Kemp Hum. Mutat. ABCD1 mutations and the X-linked adrenoleukodystrophy mutation database: role in diagnosis and clinical correlations.[Pubmed]
2001 M Biermanns Biochem. Biophys. Res. Commun. Targeting elements in the amino-terminal part direct the human 70-kDa peroxisomal integral membrane protein (PMP70) to peroxisomes.[Pubmed]
2001 E Meyhi Eur. J. Clin. Invest. Fibroblast studies documenting a case of peroxisomal 2-methylacyl-CoA racemase deficiency: possible link between racemase deficiency and malabsorption and vitamin K deficiency.[Pubmed]
2001 P Roerig FEBS Lett. Characterization and functional analysis of the nucleotide binding fold in human peroxisomal ATP binding cassette transporters.[Pubmed]
2001 Clayton Clayton Biochem. Soc. Trans. Clinical consequences of defects in peroxisomal beta-oxidation.[Pubmed]
2001 S Ferdinandusse J. Lipid Res. Plasma analysis of di- and trihydroxycholestanoic acid diastereoisomers in peroxisomal alpha-methylacyl-CoA racemase deficiency.[Pubmed]
2001 Y Suzuki J. Inherit. Metab. Dis. Clinical, biochemical and genetic aspects and neuronal migration in peroxisome biogenesis disorders.[Pubmed]
2000 L Amery J. Lipid Res. Mitochondrial and peroxisomal targeting of 2-methylacyl-CoA racemase in humans.[Pubmed]
2000 A Pujol Genomics Characterization of the adrenoleukodystrophy-related (ALDR, ABCD2) gene promoter: inductibility by retinoic acid and forskolin.[Pubmed]
2000 C Fujiwara J. Biol. Chem. Catalase-less peroxisomes. Implication in the milder forms of peroxisome biogenesis disorder.[Pubmed]
2000 T Yamada Cell Biochem. Biophys. Very-long-chain fatty acid metabolism in adrenoleukodystrophy protein-deficient mice.[Pubmed]
2000 G Unterrainer Hum. Mol. Genet. Co-expression of mutated and normal adrenoleukodystrophy protein reduces protein function: implications for gene therapy of X-linked adrenoleukodystrophy.[Pubmed]
2000 P Aubourg Glia X-linked adrenoleukodystrophy enigma: how does the ALD peroxisomal transporter mutation affect CNS glia?[Pubmed]
2000 A Gurvitz Mol. Cell Biol. Res. Commun. Adr1p-dependent regulation of the oleic acid-inducible yeast gene SPS19 encoding the peroxisomal beta-oxidation auxiliary enzyme 2,4-dienoyl-CoA reductase.[Pubmed]
2000 A Gurvitz Yeast Predicting the function and subcellular location of Caenorhabditis elegans proteins similar to Saccharomyces cerevisiae beta-oxidation enzymes.[Pubmed]
2000 P Borst Biochim. Biophys. Acta ABC transporters in lipid transport.[Pubmed]
2000 S Ferdinandusse J. Lipid Res. Subcellular localization and physiological role of alpha-methylacyl-CoA racemase.[Pubmed]
2000 K Solaas J. Lipid Res. Subcellular organization of bile acid amidation in human liver: a key issue in regulating the biosynthesis of bile salts.[Pubmed]
2000 S Ferdinandusse Nat. Genet. Mutations in the gene encoding peroxisomal alpha-methylacyl-CoA racemase cause adult-onset sensory motor neuropathy.[Pubmed]
1999 Mannaerts Mannaerts Adv. Exp. Med. Biol. Role and organization of peroxisomal beta-oxidation.[Pubmed]
1999 A Holzinger Biochem. Biophys. Res. Commun. Full length cDNA cloning, promoter sequence, and genomic organization of the human adrenoleukodystrophy related (ALDR) gene functionally redundant to the gene responsible for X-linked adrenoleukodystrophy.[Pubmed]
1999 T Imanaka J. Biol. Chem. Characterization of the 70-kDa peroxisomal membrane protein, an ATP binding cassette transporter.[Pubmed]
1999 A Netik Hum. Mol. Genet. Adrenoleukodystrophy-related protein can compensate functionally for adrenoleukodystrophy protein deficiency (X-ALD): implications for therapy.[Pubmed]
1999 M Yamasaki J. Biol. Chem. Formation of peroxisomes from peroxisomal ghosts in a peroxisome-deficient mammalian cell mutant upon complementation by protein microinjection.[Pubmed]
1999 Collins Collins Hum. Mutat. Identification of a common PEX1 mutation in Zellweger syndrome.[Pubmed]
1999 McGuinness McGuinness Arch. Immunol. Ther. Exp. (Warsz.) Cerebral inflammation in X-linked adrenoleukodystrophy.[Pubmed]
1999 Braiterman Braiterman Mol. Genet. Metab. Peroxisomal very long chain fatty acid beta-oxidation activity is determined by the level of adrenodeukodystrophy protein (ALDP) expression.[Pubmed]
1999 H Bauer Biochim. Biophys. Acta Inventory and function of yeast ABC proteins: about sex, stress, pleiotropic drug and heavy metal resistance.[Pubmed]
1999 J Berger Eur. J. Biochem. The four murine peroxisomal ABC-transporter genes differ in constitutive, inducible and developmental expression.[Pubmed]
1998 1998 1998 Verh. K. Acad. Geneeskd. Belg. New insights in peroxisomal beta-oxidation. Implications for human peroxisomal disorders.[Pubmed]
1998 R Lüers Electrophoresis Immuno-isolation of highly purified peroxisomes using magnetic beads and continuous immunomagnetic sorting.[Pubmed]
1998 S Kemp Nat. Med. Gene redundancy and pharmacological gene therapy: implications for X-linked adrenoleukodystrophy.[Pubmed]
1998 J Gärtner Eur. J. Cell Biol. Restoration of PEX2 peroxisome assembly defects by overexpression of PMP70.[Pubmed]
1998 S Braiterman Hum. Mol. Genet. Suppression of peroxisomal membrane protein defects by peroxisomal ATP binding cassette (ABC) proteins.[Pubmed]
1998 A Holzinger FEBS Lett. The mouse gene encoding the peroxisomal membrane protein 1-like protein (PXMP1-L): cDNA cloning, genomic organization and comparative expression studies.[Pubmed]
1997 K Okumoto Nat. Genet. PEX12 encodes an integral membrane protein of peroxisomes.[Pubmed]
1997 K Häyrinen Eur. J. Cell Biol. Immunocytochemical localization of the 70 kDa peroxisomal membrane protein in connections between peroxisomes in rat liver: support for a reticular organization of peroxisomes maintained by the cytoskeleton.[Pubmed]
1997 A Holzinger Biochem. Biophys. Res. Commun. Primary structure of human PMP69, a putative peroxisomal ABC-transporter.[Pubmed]
1997 N Shani Hum. Mol. Genet. Identification of a fourth half ABC transporter in the human peroxisomal membrane.[Pubmed]
1996 G Lombard-Platet Proc. Natl. Acad. Sci. U.S.A. A close relative of the adrenoleukodystrophy (ALD) gene codes for a peroxisomal protein with a specific expression pattern.[Pubmed]
1996 A Kennedy Genomics Structure and location of the murine adrenoleukodystrophy gene.[Pubmed]
1996 N Shimozawa Pediatr. Res. Correction by gene expression of biochemical abnormalities in fibroblasts from Zellweger patients.[Pubmed]
1996 T Imanaka Ann. N. Y. Acad. Sci. Sorting of the 70-kDa peroxisomal membrane protein into rat liver peroxisomes in vitro.[Pubmed]
1996 T Imanaka J. Biol. Chem. Insertion of the 70-kDa peroxisomal membrane protein into peroxisomal membranes in vivo and in vitro.[Pubmed]
1996 M Contreras Arch. Biochem. Biophys. Topology of ATP-binding domain of adrenoleukodystrophy gene product in peroxisomes.[Pubmed]
1996 N Shani Proc. Natl. Acad. Sci. U.S.A. A Saccharomyces cerevisiae homolog of the human adrenoleukodystrophy transporter is a heterodimer of two half ATP-binding cassette transporters.[Pubmed]
1996 B Hettema EMBO J. The ABC transporter proteins Pat1 and Pat2 are required for import of long-chain fatty acids into peroxisomes of Saccharomyces cerevisiae.[Pubmed]
1996 N Shani J. Biol. Chem. Characterization and analysis of conserved motifs in a peroxisomal ATP-binding cassette transporter.[Pubmed]
1995 W Schmitz Eur. J. Biochem. Purification and characterization of an alpha-methylacyl-CoA racemase from human liver.[Pubmed]
1995 F Watkins Am. J. Hum. Genet. Altered expression of ALDP in X-linked adrenoleukodystrophy.[Pubmed]
1995 N Shani Proc. Natl. Acad. Sci. U.S.A. PXA1, a possible Saccharomyces cerevisiae ortholog of the human adrenoleukodystrophy gene.[Pubmed]
1994 J Gärtner J. Inherit. Metab. Dis. Restoration of peroxisome biogenesis in a peroxisome-deficient mammalian cell line by expression of either the 35 kDa or the 70 kDa peroxisomal membrane proteins.[Pubmed]
1994 P Aubourg Curr. Opin. Genet. Dev. Adrenoleukodystrophy and other peroxisomal diseases.[Pubmed]
1994 J Mosser Hum. Mol. Genet. The gene responsible for adrenoleukodystrophy encodes a peroxisomal membrane protein.[Pubmed]
1993 J Mosser Nature Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters.[Pubmed]
1993 J Gärtner Semin. Cell Biol. The 70 kDa peroxisomal membrane protein: an ATP-binding cassette transporter protein involved in peroxisome biogenesis.[Pubmed]
1992 J Gärtner Nat. Genet. Mutations in the 70K peroxisomal membrane protein gene in Zellweger syndrome.[Pubmed]
1991 N Usuda J. Histochem. Cytochem. Immunoelectron microscopic evidence for organ differences in the composition of peroxisome-specific membrane polypeptides among three rat organs: liver, kidney, and small intestine.[Pubmed]
0 Neurology ABCD1 translation-initiator mutation demonstrates genotype-phenotype correlation for AMN.[Pubmed]
0 Mol. Membr. Biol. Trypanosoma brucei glycosomal ABC transporters: identification and membrane targeting.[Pubmed]
0 Mol. Genet. Metab. X-linked adrenoleukodystrophy: role of very long-chain acyl-CoA synthetases.[Pubmed]