ABCD3

Summary

Gene Symbol: ABCD3
Description: ATP binding cassette subfamily D member 3
Alias: ABC43, CBAS5, PMP70, PXMP1, ZWS2, ATP-binding cassette sub-family D member 3, 70 kDa peroxisomal membrane protein, ATP-binding cassette, sub-family D (ALD), member 3, Peroxisomal membrane protein-1 (70kD), dJ824O18.1 (ATP-binding cassette, sub-family D (ALD), member 3 (PMP70, PXMP1)), peroxisomal membrane protein 1 (70kD, Zellweger syndrome)
Species: human
Products:     ABCD3

Top Publications

  1. South S, Gould S. Peroxisome synthesis in the absence of preexisting peroxisomes. J Cell Biol. 1999;144:255-66 pubmed
  2. Liu L, Janvier K, Berteaux Lecellier V, Cartier N, Benarous R, Aubourg P. Homo- and heterodimerization of peroxisomal ATP-binding cassette half-transporters. J Biol Chem. 1999;274:32738-43 pubmed
    ..adrenoleukodystrophy protein (ALDP), adrenoleukodystrophy-related protein (ALDRP), and 70-kDa peroxisomal protein (PMP70) belong to the superfamily of ATP-binding cassette (ABC) transporters...
  3. Sacksteder K, Jones J, South S, Li X, Liu Y, Gould S. PEX19 binds multiple peroxisomal membrane proteins, is predominantly cytoplasmic, and is required for peroxisome membrane synthesis. J Cell Biol. 2000;148:931-44 pubmed
    ..This hypothesis is supported by the observation that the loss of PEX19 results in degradation of PMPs and/or mislocalization of PMPs to the mitochondrion. ..
  4. Brosius U, Dehmel T, Gartner J. Two different targeting signals direct human peroxisomal membrane protein 22 to peroxisomes. J Biol Chem. 2002;277:774-84 pubmed
    ..In addition, we observed that fusing the green fluorescent protein immediately adjacent to the targeting region completely abolishes targeting function and mislocalizes PMP22 to the cytosol. ..
  5. Tanaka A, Tanabe K, Morita M, Kurisu M, Kasiwayama Y, Matsuo M, et al. ATP binding/hydrolysis by and phosphorylation of peroxisomal ATP-binding cassette proteins PMP70 (ABCD3) and adrenoleukodystrophy protein (ABCD1). J Biol Chem. 2002;277:40142-7 pubmed
    The 70-kDa peroxisomal membrane protein (PMP70) and adrenoleukodystrophy protein (ALDP), half-size ATP-binding cassette transporters, are involved in metabolic transport of long and very long chain fatty acids into peroxisomes...
  6. Hillebrand M, Verrier S, Ohlenbusch A, Schäfer A, Soling H, Wouters F, et al. Live cell FRET microscopy: homo- and heterodimerization of two human peroxisomal ABC transporters, the adrenoleukodystrophy protein (ALDP, ABCD1) and PMP70 (ABCD3). J Biol Chem. 2007;282:26997-7005 pubmed
    The adrenoleukodystrophy protein (ALDP) and the 70-kDa peroxisomal membrane protein (PMP70) are half-ATP-binding cassette (ABC) transporters in the mammalian peroxisome membrane...
  7. Matsukawa T, Asheuer M, Takahashi Y, Goto J, Suzuki Y, Shimozawa N, et al. Identification of novel SNPs of ABCD1, ABCD2, ABCD3, and ABCD4 genes in patients with X-linked adrenoleukodystrophy (ALD) based on comprehensive resequencing and association studies with ALD phenotypes. Neurogenetics. 2011;12:41-50 pubmed publisher
    ..ABCD1 and ABCD2 were analyzed using a newly developed microarray-based resequencing system. ABCD3 and ABCD4 were analyzed by direct nucleotide sequence analysis...
  8. Biermanns M, Gartner J. Targeting elements in the amino-terminal part direct the human 70-kDa peroxisomal integral membrane protein (PMP70) to peroxisomes. Biochem Biophys Res Commun. 2001;285:649-55 pubmed
    ..The 70-kDa peroxisomal integral membrane protein (PMP70) is one of the proteins located in the human peroxisome membrane...
  9. Debbabi M, Nury T, Zarrouk A, Mekahli N, Bezine M, Sghaier R, et al. Protective Effects of ?-Tocopherol, ?-Tocopherol and Oleic Acid, Three Compounds of Olive Oils, and No Effect of Trolox, on 7-Ketocholesterol-Induced Mitochondrial and Peroxisomal Dysfunction in Microglial BV-2 Cells. Int J Mol Sci. 2016;17: pubmed
    ..In addition, ?-and ?-tocopherol, and OA were able to prevent the decrease in Abcd3 protein levels, which allows the measurement of peroxisomal mass, and in mRNA levels of Abcd1 and Abcd2, which ..

More Information

Publications109 found, 100 shown here

  1. Fu G, Wang L, Li L, Liu J, Liu S, Zhao X. Bacillus licheniformis CK1 alleviates the toxic effects of zearalenone in feed on weaned female Tibetan piglets. J Anim Sci. 2018;96:4471-4480 pubmed publisher
    ..cassette (ABC) transporters: ABCB1 and ABCb4 in the vagina, ABCA1 and ABCb4 in the uterus, and ABCB1, ABCb4, ABCD3, and ABCG2 in the ovary, while these transporters in the T2 group were significantly decreased (P < 0...
  2. Zaabi N, Kendi A, Al Jasmi F, Takashima S, Shimozawa N, Al Dirbashi O. Atypical PEX16 peroxisome biogenesis disorder with mild biochemical disruptions and long survival. Brain Dev. 2019;41:57-65 pubmed publisher
    ..Immunocytochemical investigations on fibroblasts revealed fewer catalase and PMP70-containing particles indicating aberrant peroxisomal assembly. Complementation studies were inconclusive...
  3. Miura S, Kosaka K, Nomura T, Nagata S, Shimojo T, Morikawa T, et al. TDRKH is a candidate gene for an autosomal dominant distal hereditary motor neuropathy. Eur J Med Genet. 2018;: pubmed publisher
    ..851g > a [p.arg284his] in tdrkh, nm_002858: c.1654g > t [p.gly552cys] in abcd3, nm_001005164: c.898a > t [p.ile300phe], in or52e2)...
  4. Dai Q, Sun W, Xiong Y, Hankey G, Xiao L, Zhu W, et al. From clinical to tissue-based dual TIA: Validation and refinement of ABCD3-I score. Neurology. 2015;84:1426-32 pubmed publisher
    ..18, 95% confidence interval 0.69-2.01). C statistics was higher when the item of dual TIA in ABCD3-I score was replaced by dual DWI (0.759 vs 0.729, p = 0.035)...
  5. De Marchis G, Weck A, Audebert H, Benik S, Foerch C, Buhl D, et al. Copeptin for the prediction of recurrent cerebrovascular events after transient ischemic attack: results from the CoRisk study. Stroke. 2014;45:2918-23 pubmed publisher
    ..with the ABCD2 (age, blood, clinical features of TIA, duration of symptoms, presence of diabetes mellitus) and ABCD3-I (ABCD2, dual TIA [the presence of ≥2 TIA symptoms within 7 days], imaging [the presence of abnormal ..
  6. Uyama T, Kawai K, Kono N, Watanabe M, Tsuboi K, Inoue T, et al. Interaction of Phospholipase A/Acyltransferase-3 with Pex19p: A POSSIBLE INVOLVEMENT IN THE DOWN-REGULATION OF PEROXISOMES. J Biol Chem. 2015;290:17520-34 pubmed publisher
    ..24 h, as revealed by diffuse signals of DsRed2-Peroxi and a remarkable decrease in a peroxisomal membrane protein, PMP70. A time-dependent decrease in ether-type lipid levels was also seen...
  7. Kawaguchi K, Morita M. ABC Transporter Subfamily D: Distinct Differences in Behavior between ABCD1-3 and ABCD4 in Subcellular Localization, Function, and Human Disease. Biomed Res Int. 2016;2016:6786245 pubmed
    ..chain fatty acids (VLCFA) or their CoA-derivatives into peroxisomes with different substrate specificities, while ABCD3 is involved in the transport of branched chain acyl-CoA into peroxisomes...
  8. Kemp S, Wanders R. X-linked adrenoleukodystrophy: very long-chain fatty acid metabolism, ABC half-transporters and the complicated route to treatment. Mol Genet Metab. 2007;90:268-76 pubmed
    ..The peroxisomal membrane harbors three additional closely related ABC half-transporters, ALDRP, PMP70 and PMP69 (PMP70R). ABC half-transporters must dimerize to form a functional full-transporter...
  9. Woudenberg J, Rembacz K, van den Heuvel F, Woudenberg Vrenken T, Buist Homan M, Geuken M, et al. Caveolin-1 is enriched in the peroxisomal membrane of rat hepatocytes. Hepatology. 2010;51:1744-53 pubmed publisher
    ..analyses reveal that caveolin-1 colocalizes with peroxisomal marker proteins (catalase, the 70 kDa peroxisomal membrane protein PMP70, the adrenoleukodystrophy protein ALDP, Pex14p, and the bile acid-coenzyme A:amino acid N-..
  10. Soliman K, Göttfert F, Rosewich H, Thoms S, Gartner J. Super-resolution imaging reveals the sub-diffraction phenotype of Zellweger Syndrome ghosts and wild-type peroxisomes. Sci Rep. 2018;8:7809 pubmed publisher
    ..Further, we investigated the 70?kDa peroxisomal membrane protein (PMP70) abundance in relationship to the ZSS sub-diffraction phenotype...
  11. Nury T, Sghaier R, Zarrouk A, Menetrier F, Uzun T, Leoni V, et al. Induction of peroxisomal changes in oligodendrocytes treated with 7-ketocholesterol: Attenuation by α-tocopherol. Biochimie. 2018;153:181-202 pubmed publisher
    ..with abnormal sizes and shapes similar to those observed in Zellweger fibroblasts, lower cellular level of ABCD3, used as a marker of peroxisomal mass, measured by flow cytometry, lower mRNA and protein levels (measured by RT-..
  12. Tang Y, M Vanlandingham M, Wu Y, Beland F, Olson G, Fang J. Role of peroxisome proliferator-activated receptor alpha (PPARα) and PPARα-mediated species differences in triclosan-induced liver toxicity. Arch Toxicol. 2018;92:3391-3402 pubmed publisher
    ..Triclosan also elevated the expression levels of peroxisomal membrane protein PMP70 and catalase in the livers of both genotypes, suggesting that triclosan promoted the production of hepatocyte ..
  13. Geuze H, Murk J, Stroobants A, Griffith J, Kleijmeer M, Koster A, et al. Involvement of the endoplasmic reticulum in peroxisome formation. Mol Biol Cell. 2003;14:2900-7 pubmed
    ..We found the peroxisomal membrane protein Pex13p and the ATP-binding cassette transporter protein PMP70 present in specialized subdomains of the ER that were continuous with a peroxisomal reticulum from which mature ..
  14. Porcellotti S, Fanelli F, Fracassi A, Sepe S, Cecconi F, Bernardi C, et al. Oxidative Stress during the Progression of β-Amyloid Pathology in the Neocortex of the Tg2576 Mouse Model of Alzheimer's Disease. Oxid Med Cell Longev. 2015;2015:967203 pubmed publisher
    ..At 6 months, when intracellular Aβ accumulates, PMP70 is downregulated, indicating impairment of fatty acids peroxisomal translocation and their consequent harmful ..
  15. Zhao M, Wang S, Zhang D, Zhang Y, Deng X, Zhao J. Comparison of Stroke Prediction Accuracy of ABCD2 and ABCD3-I in Patients with Transient Ischemic Attack: A Meta-Analysis. J Stroke Cerebrovasc Dis. 2017;26:2387-2395 pubmed publisher
    A direct comparison of the stroke prediction utility of the ABCD2 and ABCD3-I scores has not been performed...
  16. Baarine M, Ragot K, Genin E, El Hajj H, Trompier D, Andreoletti P, et al. Peroxisomal and mitochondrial status of two murine oligodendrocytic cell lines (158N, 158JP): potential models for the study of peroxisomal disorders associated with dysmyelination processes. J Neurochem. 2009;111:119-31 pubmed publisher
    ..CNPase and myelin oligodendrocyte glycoprotein), and peroxisomal markers [adrenoleukodystrophy protein, PMP70, acyl-CoA oxidase 1 (ACOX1), l-peroxisomal bifunctional enzyme, and catalase]...
  17. Karnati S, Palaniswamy S, Alam M, Oruqaj G, Stamme C, Baumgart Vogt E. C22-bronchial and T7-alveolar epithelial cell lines of the immortomouse are excellent murine cell culture model systems to study pulmonary peroxisome biology and metabolism. Histochem Cell Biol. 2016;145:287-304 pubmed publisher
    ..catalase and antioxidative enzymes in distinct subcellular compartments, whereas T7 cells exhibit higher levels of ABCD3 and plasmalogen synthesizing enzymes as well as nuclear receptors of the PPAR family...
  18. Kang Y, Park S, Ahn C, Song J, Kim D, Jin E. Beneficial reward-to-risk action of glucosamine during pathogenesis of osteoarthritis. Eur J Med Res. 2015;20:89 pubmed publisher
    ..Peroxisomal function was analyzed by BODIPY staining, and gene expression of PMP70 and acyl-CoA oxidase 1, by real-time PCR. Total lipids were analyzed by gas chromatography/mass spectrometry...
  19. Lee J, Dutta R, Maharjan Y, Liu Z, Lim J, Kim S, et al. Catalase inhibition induces pexophagy through ROS accumulation. Biochem Biophys Res Commun. 2018;501:696-702 pubmed publisher
    ..3AT) decreased the number of peroxisomes and resulted in the downregulation of peroxisomal proteins, such as PMP70 and PEX14 under serum starvation...
  20. Nury T, Zarrouk A, Ragot K, Debbabi M, Riedinger J, Vejux A, et al. 7-Ketocholesterol is increased in the plasma of X-ALD patients and induces peroxisomal modifications in microglial cells: Potential roles of 7-ketocholesterol in the pathophysiology of X-ALD. J Steroid Biochem Mol Biol. 2017;169:123-136 pubmed publisher
    ..7KC also induces several peroxisomal modifications: decreased Abcd1, Abcd2, Abcd3, Acox1 and/or Mfp2 mRNA and protein levels, increased catalase activity and decreased Acox1-activity...
  21. Ilieva H, Nagano I, Murakami T, Shiote M, Manabe Y, Abe K. Age-related changes in peroxisomal membrane protein 70 and superoxide dismutase 1 in transgenic G93A mice. Neurol Res. 2003;25:423-6 pubmed
    Peroxisomal membrane protein 70 (PMP70) and Cu/Zn superoxide dismutase (SOD1) were examined in the spinal cords of transgenic (Tg) mice expressing a human mutant SOD1 protein (G93A) and their age-matched controls at 8, 20 and 32 weeks by ..
  22. Bai P, Csóka B. New route for the activation of poly(ADP-ribose) polymerase-1: a passage that links poly(ADP-ribose) polymerase-1 to lipotoxicity?. Biochem J. 2015;469:e9-11 pubmed publisher
    ..This study envisages a pathway through which ABCD3 translates enhanced fatty acid levels to ERK and consequently PARP-1 activation...
  23. Sychev Z, Hu A, Dimaio T, Gitter A, Camp N, Noble W, et al. Integrated systems biology analysis of KSHV latent infection reveals viral induction and reliance on peroxisome mediated lipid metabolism. PLoS Pathog. 2017;13:e1006256 pubmed publisher
    ..Additionally, proteins involved in peroxisomal lipid metabolism of very long chain fatty acids, including ABCD3 and ACOX1, are required for the survival of latently infected cells...
  24. Gartner J, Kearns W, Rosenberg C, Pearson P, Copeland N, Gilbert D, et al. Localization of the 70-kDa peroxisomal membrane protein to human 1p21-p22 and mouse 3. Genomics. 1993;15:412-4 pubmed
    The 70-kDa peroxisomal membrane protein (PXMP1) is a member of the ATP-binding cassette transporter family...
  25. Jones J, Morrell J, Gould S. PEX19 is a predominantly cytosolic chaperone and import receptor for class 1 peroxisomal membrane proteins. J Cell Biol. 2004;164:57-67 pubmed
  26. Lee J, Kim S, Lim J, Kim S, Choe S, Park R. Proteasome inhibitors induce auditory hair cell death through peroxisome dysfunction. Biochem Biophys Res Commun. 2015;456:269-74 pubmed publisher
    ..inhibitor significantly decreases both the number of peroxisomes and expression of peroxisomal proteins such as PMP70 and Catalase...
  27. Esmaeili M, Ghaedi K, Shoaraye Nejati A, Nematollahi M, Shiralyian H, Nasr Esfahani M. Pioglitazone significantly prevented decreased rate of neural differentiation of mouse embryonic stem cells which was reduced by Pex11β knock-down. Neuroscience. 2016;312:35-47 pubmed publisher
    ..Additionally, mRNA levels of other peroxisome-related genes such as PMP70, Pex11α, Catalase, Pex19 and Pex5 were also significantly reduced by Pex11β knock-down (p<0.05)...
  28. Debbabi M, Nury T, Helali I, Karym E, Geillon F, Gondcaille C, et al. Flow Cytometric Analysis of the Expression Pattern of Peroxisomal Proteins, Abcd1, Abcd2, and Abcd3 in BV-2 Murine Microglial Cells. Methods Mol Biol. 2017;1595:257-265 pubmed publisher
    ..So, the expression pattern of various peroxisomal transporters (Abcd1, Abcd2, Abcd3) contributing to peroxisomal ?-oxidation was evaluated on BV-2 cells by flow cytometry and complementary methods (..
  29. Mayer L, Ferrari J, Krebs S, Boehme C, Toell T, Matosevic B, et al. ABCD3-I score and the risk of early or 3-month stroke recurrence in tissue- and time-based definitions of TIA and minor stroke. J Neurol. 2018;265:530-534 pubmed publisher
    Changing definition of TIA from time to a tissue basis questions the validity of the well-established ABCD3-I risk score for recurrent ischemic cerebrovascular events...
  30. Iwashita S, Tsuchida M, Tsukuda M, Yamashita Y, Emi Y, Kida Y, et al. Multiple organelle-targeting signals in the N-terminal portion of peroxisomal membrane protein PMP70. J Biochem. 2010;147:581-90 pubmed publisher
    ..organelle-targeting properties of the N-terminal portions of the peroxisomal isoform of the ABC transporter PMP70 (ABCD3) using enhanced green fluorescent protein (EGFP) fusion...
  31. Baarine M, Ragot K, Athias A, Nury T, Kattan Z, Genin E, et al. Incidence of Abcd1 level on the induction of cell death and organelle dysfunctions triggered by very long chain fatty acids and TNF-? on oligodendrocytes and astrocytes. Neurotoxicology. 2012;33:212-28 pubmed publisher
    ..a destabilization of lysosomal membrane and a cytoplasmic redistribution of lysosomes; (iii) a modulation of Abcd3/PMP70 and Acox-1 protein expression, and a decrease in catalase activity at the peroxisomal level...
  32. Jin A, Lee J, Kim M, Kwak S, Kim S, Song K, et al. 2,2'-dipyridyl induces pexophagy. Biochem Biophys Res Commun. 2016;469:941-7 pubmed publisher
    ..originating from peroxisomes and a decrease in the expression of peroxisomal proteins such as catalase, Pex14, and PMP70. The phenotype was defined as pexophagy, because 2,2-DP induced autophagy and inhibition of autophagy ..
  33. Kelly P, Albers G, Chatzikonstantinou A, De Marchis G, Ferrari J, George P, et al. Validation and comparison of imaging-based scores for prediction of early stroke risk after transient ischaemic attack: a pooled analysis of individual-patient data from cohort studies. Lancet Neurol. 2016;15:1238-1247 pubmed publisher
    ..We compared the prognostic utility of the ABCD2, ABCD2-I, and ABCD3-I scores using discrimination, calibration, and risk reclassification...
  34. Dahabieh M, Ha Z, Di Pietro E, Nichol J, Bolt A, Goncalves C, et al. Peroxisomes protect lymphoma cells from HDAC inhibitor-mediated apoptosis. Cell Death Differ. 2017;24:1912-1924 pubmed publisher
    ..numbers in cultured lymphoma cells, concomitant with increased levels of peroxisomal proteins PEX3, PEX11B, and PMP70. Genetic inhibition of peroxisomes, using PEX3 knockdown, reveals that peroxisomes protect lymphoma cells against ..
  35. Imanaka T, Aihara K, Suzuki Y, Yokota S, Osumi T. The 70-kDa peroxisomal membrane protein (PMP70), an ATP-binding cassette transporter. Cell Biochem Biophys. 2000;32 Spring:131-8 pubmed
    The 70-kDa peroxisomal membrane protein (PMP70) is one of major components of peroxisomal membranes...
  36. Yasui K, Mihara S, Zhao C, Okamoto H, Saito Ohara F, Tomida A, et al. Alteration in copy numbers of genes as a mechanism for acquired drug resistance. Cancer Res. 2004;64:1403-10 pubmed
    ..cassette (ABC) transporter genes [ABCA3, ABCB1 (MDR1), ABCB6, ABCB8, ABCB10, ABCB11, ABCC1 (MRP1), ABCC4, ABCC9, ABCD3, ABCD4, ABCE1, and ABCF2] were amplified among 19 of the resistant cell lines examined...
  37. Hirai T, Fukui Y, Motojima K. PPARalpha agonists positively and negatively regulate the expression of several nutrient/drug transporters in mouse small intestine. Biol Pharm Bull. 2007;30:2185-90 pubmed
    ..Expression levels of seven nutrient/drug transporters (Abcd3, Octn2/Slc22a5, FATP2/Slc27a2, Slc22a21, Mct13/Slc16a13, Slc23a1 and Bcrp/Abcg2) in the intestine were up-..
  38. Shibata H, Kashiwayama Y, Imanaka T, Kato H. Domain architecture and activity of human Pex19p, a chaperone-like protein for intracellular trafficking of peroxisomal membrane proteins. J Biol Chem. 2004;279:38486-94 pubmed
    ..In a similar manner, Pex19p was bound to PMP70 and Pex16p as well as the Pex3p soluble fragment...
  39. Kashiwayama Y, Asahina K, Shibata H, Morita M, Muntau A, Roscher A, et al. Role of Pex19p in the targeting of PMP70 to peroxisome. Biochim Biophys Acta. 2005;1746:116-28 pubmed
    Pex19p is a protein required for the peroxisomal membrane synthesis. The 70-kDa peroxisomal membrane protein (PMP70) is synthesized on free cytosolic ribosomes and then inserted posttranslationally into peroxisomal membranes...
  40. Reams R, Jones Triche J, Chan O, Hernandez B, Soliman K, Yates C. Immunohistological analysis of ABCD3 expression in Caucasian and African American prostate tumors. Biomed Res Int. 2015;2015:132981 pubmed publisher
    In a previously published study, we showed that expression of the ABCD3 gene increased with increasing metastatic potential in a panel of prostate cancer cell lines derived from African American and Caucasian American men...
  41. Liu Y, Pu Q, Wu M, Yu C. Proteomic analysis for the impact of hypercholesterolemia on expressions of hepatic drug transporters and metabolizing enzymes. Xenobiotica. 2016;46:940-7 pubmed publisher
    ..Among those, protein levels of hepatic drug transporters (MRP2, ABCD3, OAT2, SLC25A12, SCL38A3, SLC2A2 and SLC25A5) and metabolizing enzymes (CYP2B3, CYP2C7, CYP2C11, CYP2C13, CYP4A2 ..
  42. Gartner J, Moser H, Valle D. Mutations in the 70K peroxisomal membrane protein gene in Zellweger syndrome. Nat Genet. 1992;1:16-23 pubmed
    The peroxisomal membrane protein, with a relative molecular mass of 70,000 (M(r) 70K) (PMP70), is an important component of peroxisomal membranes and an ATP-binding cassette protein...
  43. Iida A, Saito S, Sekine A, Mishima C, Kitamura Y, Kondo K, et al. 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. J Hum Genet. 2002;47:285-310 pubmed
    ..13 genes belonging to the superfamily of ATP-binding cassette transporters ( ABCA4, ABCA7, ABCA8, ABCD1, ABCD3, ABCD4, ABCE1, ABCF1, ABCG1, ABCG2, ABCG4, ABCG5, and ABCG8)...
  44. Knoflach M, Lang W, Seyfang L, Fertl E, Oberndorfer S, Daniel G, et al. Predictive value of ABCD2 and ABCD3-I scores in TIA and minor stroke in the stroke unit setting. Neurology. 2016;87:861-9 pubmed publisher
    ..ABCD2 and ABCD3-I scores have been prospectively documented in a cohort of patients admitted to Austrian stroke units within 24 ..
  45. Huang T, Zheng D, Houmard J, Brault J, Hickner R, Cortright R. Overexpression of PGC-1α increases peroxisomal activity and mitochondrial fatty acid oxidation in human primary myotubes. Am J Physiol Endocrinol Metab. 2017;312:E253-E263 pubmed publisher
    ..After overexpression of PGC-1α, 1) peroxisomal membrane protein 70 kDa (PMP70), PEX19, and mitochondrial citrate synthetase protein content were significantly elevated (P < 0...
  46. Morvay P, Baes M, Van Veldhoven P. Differential activities of peroxisomes along the mouse intestinal epithelium. Cell Biochem Funct. 2017;35:144-155 pubmed publisher
    ..Other peroxisomal enzymes (PHYH, HACL1, and ACAA1), the ABCD2 and ABCD3 transporters, and peroxins PEX13 and PEX14, however, did not follow this pattern, displaying rather constant ..
  47. Kamijo K, Kamijo T, Ueno I, Osumi T, Hashimoto T. Nucleotide sequence of the human 70 kDa peroxisomal membrane protein: a member of ATP-binding cassette transporters. Biochim Biophys Acta. 1992;1129:323-7 pubmed
    The cDNA sequence of human liver 70 kDa peroxisomal membrane protein (hPMP70) was determined. The nucleotide sequence contains an open reading frame of 1977 base pairs and encodes an amino acid sequence of 659 residues which exhibits 95...
  48. Braiterman L, Zheng S, Watkins P, Geraghty M, Johnson G, McGuinness M, et al. Suppression of peroxisomal membrane protein defects by peroxisomal ATP binding cassette (ABC) proteins. Hum Mol Genet. 1998;7:239-47 pubmed
    ..ALDP and three other ABC proteins (PMP70, ALDR, P70R) localize to the peroxisomal membrane...
  49. Asheuer M, Bieche I, Laurendeau I, Moser A, Hainque B, Vidaud M, et al. Decreased expression of ABCD4 and BG1 genes early in the pathogenesis of X-linked adrenoleukodystrophy. Hum Mol Genet. 2005;14:1293-303 pubmed
    ..of ALD, we studied the expression of ABCD1, three other peroxisomal transporter genes of the same family (ABCD2, ABCD3 and ABCD4) and two VLCFA synthetase genes (VLCS and BG1) involved in VLCFA metabolism, as well as the VLCFA ..
  50. Gueugnon F, Gondcaille C, Leclercq S, Bellenger J, Bellenger S, Narce M, et al. Dehydroepiandrosterone up-regulates the Adrenoleukodystrophy-related gene (ABCD2) independently of PPARalpha in rodents. Biochimie. 2007;89:1312-21 pubmed
    ..We show that Abcd2 and Abcd3 but not Abcd4 are induced in primary culture of rat hepatocytes by DHEA-S...
  51. Liu X, Liu J, Lester J, Pijut S, Graf G. ABCD2 identifies a subclass of peroxisomes in mouse adipose tissue. Biochem Biophys Res Commun. 2015;456:129-34 pubmed publisher
    ..generated a fraction enriched with D2, but deficient in peroxisome markers including catalase, PEX19, and ABCD3 (D3)...
  52. Kenn M, Ribarics R, Ilieva N, Cibena M, Karch R, Schreiner W. Spatiotemporal multistage consensus clustering in molecular dynamics studies of large proteins. Mol Biosyst. 2016;12:1600-14 pubmed publisher
    ..Three alleles of the MHC, each in complex with ABCD3 peptide and LC13 T-cell receptor (TCR), yielded different patterns of motion...
  53. Lombard Platet G, Savary S, Sarde C, Mandel J, Chimini G. A close relative of the adrenoleukodystrophy (ALD) gene codes for a peroxisomal protein with a specific expression pattern. Proc Natl Acad Sci U S A. 1996;93:1265-9 pubmed
    ..ALDP showed 38% sequence identity with another peroxisomal membrane protein, PMP70, up to now its closest homologue...
  54. Shimozawa N, Suzuki Y, Tomatsu S, Tsukamoto T, Osumi T, Fujiki Y, et al. Correction by gene expression of biochemical abnormalities in fibroblasts from Zellweger patients. Pediatr Res. 1996;39:812-5 pubmed
    ..these disorders, and mutations in peroxisome assembly factor-1 (PAF-1) and the 70-kD peroxisomal membrane protein (PMP70) genes have been detected by our group F and Roscher's group 1, respectively...
  55. Shani N, Valle D. A Saccharomyces cerevisiae homolog of the human adrenoleukodystrophy transporter is a heterodimer of two half ATP-binding cassette transporters. Proc Natl Acad Sci U S A. 1996;93:11901-6 pubmed
    The adrenoleukodystrophy protein (ALDP) and the 70-kDa peroxisomal membrane protein (PMP70) are half ATP-binding cassette (ABC) transporters in the human peroxisome membrane...
  56. Gartner J, Jimenez Sanchez G, Roerig P, Valle D. Genomic organization of the 70-kDa peroxisomal membrane protein gene (PXMP1). Genomics. 1998;48:203-8 pubmed
    The 70-kDa peroxisomal membrane protein (PMP70) is a member of a family of half-ATP-binding cassette (ABC) transporter proteins located in the human peroxisomal membrane...
  57. Holzinger A, Muntau A, Mayerhofer P, Kammerer S, Albet S, Bugaut M, et al. The mouse gene encoding the peroxisomal membrane protein 1-like protein (PXMP1-L): cDNA cloning, genomic organization and comparative expression studies. FEBS Lett. 1998;433:179-83 pubmed
    ..Its closest homolog is the peroxisomal membrane protein 1 (PMP70). We have cloned the mouse PXMP1-L gene. It encodes a 606 amino acid protein...
  58. Collins C, Gould S. Identification of a common PEX1 mutation in Zellweger syndrome. Hum Mutat. 1999;14:45-53 pubmed
    ..Furthermore, mutations in the PMP70 gene have also been identified in two Zellweger syndrome patients from CG1, raising the possibility that CG1 ..
  59. Berger J, Albet S, Bentejac M, Netik A, Holzinger A, Roscher A, et al. The four murine peroxisomal ABC-transporter genes differ in constitutive, inducible and developmental expression. Eur J Biochem. 1999;265:719-27 pubmed
    ..protein (ALDP), adrenoleukodystrophy-related protein (ALDRP), 70-kDa peroxisomal membrane protein (PMP70) and PMP70-related protein (P70R)...
  60. Wilcke M, Alexson S. Differential induction of peroxisomal populations in subcellular fractions of rat liver. Biochim Biophys Acta. 2001;1544:358-69 pubmed
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    ..ABCD1, and the two close homologues ABCD2 (ALDR) and ABCD3 (PMP70), are genes encoding ATP-binding cassette half-transporters of the peroxisomal membrane...
  62. Mayerhofer P, Kattenfeld T, Roscher A, Muntau A. Two splice variants of human PEX19 exhibit distinct functions in peroxisomal assembly. Biochem Biophys Res Commun. 2002;291:1180-6 pubmed
    ..Both interact with peroxisomal ABC transporters (ALDP, ALDRP, PMP70) and with full-length PEX3 as shown by in vitro protein interaction studies...
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    ALDP, ALDPR, PMP70 and PMP70R are half ATP-binding cassette (ABC) transporters of the mammalian peroxisomal membrane...
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    ..The availability of mutant mice in which Abcd1, Abcd2, or Abcd3 have been disrupted will help to resolve the true role of the peroxisomal half-ABC transporters.
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    ..Further, the region between transmembrane domain 2 and 3 is important for the targeting of ALDP to the peroxisome...
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    ..3alpha-diol) on the expression of the ABC half-transporters encoded by the ABCD2 and ABCD3 genes, in fibroblasts drawn from controls and from two affected brothers...
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    ..antibodies, we found that residual ?-oxidation activity toward C26:0-CoA in X-ALD fibroblasts is mediated by ABCD3, although the efficacy of ABCD3 appeared to be much lower than that of ABCD1...
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    ..17% amino acid substitution per million years) than PMP35 (0.14%), PMP70 (0.07%), or catalase genes (0.13%)...
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    The 70 kDa peroxisomal membrane protein (PMP70) is a major component of peroxisomal membranes. cDNAs for human and rat PMP70 have been cloned and sequenced and the gene mapped to human chromosome 1p21-22...
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    ..blot analysis showed the absence of ALDP in the brain, spinal cord, lung, and kidney and normal expression of PMP70 in the liver, lung, and kidney...
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    Three half ATP-binding cassette transporters (ALDP, ALDR, PMP70) are known to be present in the human peroxisome membrane...
  75. Imanaka T, Aihara K, Takano T, Yamashita A, Sato R, Suzuki Y, et al. Characterization of the 70-kDa peroxisomal membrane protein, an ATP binding cassette transporter. J Biol Chem. 1999;274:11968-76 pubmed
    The 70-kDa peroxisomal membrane protein (PMP70) is one of the major components of rat liver peroxisomal membranes and belongs to a superfamily of proteins known as ATP binding cassette transporters...
  76. Gloeckner C, Mayerhofer P, Landgraf P, Muntau A, Holzinger A, Gerber J, et al. Human adrenoleukodystrophy protein and related peroxisomal ABC transporters interact with the peroxisomal assembly protein PEX19p. Biochem Biophys Res Commun. 2000;271:144-50 pubmed
    Four ABC half transporters (ALDP, ALDRP, PMP70, and PMP69) have been identified in the mammalian peroxisomal membrane but no function has been unambiguously assigned to any of them...
  77. Schroeder F, Frolov A, Starodub O, Atshaves B, Russell W, Petrescu A, et al. Pro-sterol carrier protein-2: role of the N-terminal presequence in structure, function, and peroxisomal targeting. J Biol Chem. 2000;275:25547-55 pubmed
    ..cDNA encoding pro-SCP-2 resulted in 45% and 59% of SCP-2, respectively, colocalizing with the peroxisomal marker PMP70. In contrast, l-cells transfected with cDNA encoding SCP-2 exhibited 3-fold lower colocalization of SCP-2 with ..
  78. Fujiwara C, Imamura A, Hashiguchi N, Shimozawa N, Suzuki Y, Kondo N, et al. Catalase-less peroxisomes. Implication in the milder forms of peroxisome biogenesis disorder. J Biol Chem. 2000;275:37271-7 pubmed
    ..33 degrees C), catalase gradually localized to the structures containing a 70-kDa peroxisomal membrane protein, PMP70. In contrast to catalase, other matrix proteins containing typical peroxisome targeting signals, acyl-CoA oxidase ..
  79. Dunster K, Lai F, Sentry J. Limkain b1, a novel human autoantigen localized to a subset of ABCD3 and PXF marked peroxisomes. Clin Exp Immunol. 2005;140:556-63 pubmed
    ..on the identification of a novel autoantigen that co-localizes with a subset of cytoplasmic microbodies marked by ABCD3 (PMP-70) and/or PXF (PEX19)...
  80. Toro A, Araya C, Córdova G, Arredondo C, Cárdenas H, Moreno R, et al. Pex3p-dependent peroxisomal biogenesis initiates in the endoplasmic reticulum of human fibroblasts. J Cell Biochem. 2009;107:1083-96 pubmed publisher
    ..These results indicate that Pex3p follows the ER-to-peroxisomal route in mammalian cells and provides new clues to understand its function. ..
  81. Kamijo K, Taketani S, Yokota S, Osumi T, Hashimoto T. The 70-kDa peroxisomal membrane protein is a member of the Mdr (P-glycoprotein)-related ATP-binding protein superfamily. J Biol Chem. 1990;265:4534-40 pubmed
    The 70-kDa peroxisomal membrane protein (PMP70) is one of the major integral membrane proteins of rat liver peroxisomes. cDNA clones for PMP70 were isolated and sequenced...
  82. Holzinger A, Kammerer S, Roscher A. Primary structure of human PMP69, a putative peroxisomal ABC-transporter. Biochem Biophys Res Commun. 1997;237:152-7 pubmed
    ..peroxisomal ABC-half-transporters such as the adrenoleukodystrophy protein (ALDP) and the peroxisomal protein 70 (PMP70). This 2927-bp cDNA codes for a 606 aminoacid (68...
  83. Holzinger A, Roscher A, Landgraf P, Lichtner P, Kammerer S. Genomic organization and chromosomal localization of the human peroxisomal membrane protein-1-like protein (PXMP1-L) gene encoding a peroxisomal ABC transporter. FEBS Lett. 1998;426:238-42 pubmed
    The cDNA of the peroxisomal membrane protein-1-like protein (PXMP1-L, synonyms: PMP69, P70R), a novel peroxisomal ATP binding cassette transporter of yet unknown function, has recently been cloned...
  84. Gartner J, Brosius U, Obie C, Watkins P, Valle D. Restoration of PEX2 peroxisome assembly defects by overexpression of PMP70. Eur J Cell Biol. 1998;76:237-45 pubmed
    ..Surprisingly, expression of the human 70 kDa peroxisomal membrane protein (PMP70) also restores peroxisome biogenesis in these same CHO cell clones...
  85. Netik A, Forss Petter S, Holzinger A, Molzer B, Unterrainer G, Berger J. Adrenoleukodystrophy-related protein can compensate functionally for adrenoleukodystrophy protein deficiency (X-ALD): implications for therapy. Hum Mol Genet. 1999;8:907-13 pubmed
    ..peroxisomal ABC transporters currently are known: adrenoleukodystrophy-related protein (ALDRP), 70 kDa peroxisomal membrane protein (PMP70) and PMP70- related protein...
  86. Roerig P, Mayerhofer P, Holzinger A, Gartner J. Characterization and functional analysis of the nucleotide binding fold in human peroxisomal ATP binding cassette transporters. FEBS Lett. 2001;492:66-72 pubmed
    The 70-kDa peroxisomal membrane protein (PMP70) and the adrenoleukodystrophy protein (ALDP) are half ATP binding cassette (ABC) transporters in the peroxisome membrane...
  87. Kashiwayama Y, Morita M, Kamijo K, Imanaka T. Nucleotide-induced conformational changes of PMP70, an ATP binding cassette transporter on rat liver peroxisomal membranes. Biochem Biophys Res Commun. 2002;291:1245-51 pubmed
    Nucleotide-induced conformational changes of the 70-kDa peroxisomal membrane protein (PMP70) were investigated by means of limited-trypsin digestion...
  88. Morita M, Kanai M, Mizuno S, Iwashima M, Hayashi T, Shimozawa N, et al. Baicalein 5,6,7-trimethyl ether activates peroxisomal but not mitochondrial fatty acid beta-oxidation. J Inherit Metab Dis. 2008;31:442-9 pubmed publisher
    ..In addition, we found that BTM did not upregulate the expression of ABCD2/ALDR, ABCD3/PMP70, ACOX1 and FATP4 genes but slightly increased ACSVL1 gene expression.
  89. Di Benedetto R, Denti M, Salvati S, Attorri L, Di Biase A. PMP70 knock-down generates oxidative stress and pro-inflammatory cytokine production in C6 glial cells. Neurochem Int. 2009;54:37-42 pubmed publisher
    ..in rat C6 glial cells, we previously generated the cell line abcd3kd in which the peroxisomal half-transporter PMP70 was stably knocked-down...
  90. Gondcaille C, Genin E, Lopez T, Dias A, Geillon F, Andreoletti P, et al. LXR antagonists induce ABCD2 expression. Biochim Biophys Acta. 2014;1841:259-66 pubmed
    ..Two homologous peroxisomal ABC transporters, ABCD2 and ABCD3 have been proven to compensate for ABCD1 deficiency when overexpressed...
  91. van Roermund C, IJlst L, Wagemans T, Wanders R, Waterham H. A role for the human peroxisomal half-transporter ABCD3 in the oxidation of dicarboxylic acids. Biochim Biophys Acta. 2014;1841:563-8 pubmed publisher
    ..All these fatty acids are most likely transported as CoA esters. We postulate a role for human ABCD3 in the oxidation of dicarboxylic acids and a role in buffering fatty acids that are overflowing from the ..
  92. Jefferson M, Whelband M, Mohorianu I, Powell P. The pestivirus N terminal protease N(pro) redistributes to mitochondria and peroxisomes suggesting new sites for regulation of IRF3 by N(pro.). PLoS ONE. 2014;9:e88838 pubmed publisher
    ..in addition, both N(pro) and IRF3 associated with peroxisomes, with over 85% of N(pro) puncta co-distributing with PMP70, a marker for peroxisomes. In addition, peroxisomes containing N(pro) and IRF3 associated with ubiquitin...