Impaired very long-chain acyl-CoA β-oxidation in human X-linked adrenoleukodystrophy fibroblasts is a direct consequence of ABCD1 transporter dysfunction

Journal of Biological Chemistry

Volumn 288, Issue 26, 2013, Pages 19269-19279

  Wiesinger, Christoph ^a   Kunze, Markus ^a   Regelsberger, Günther ^b   Forss Petter, Sonja ^a   Berger, Johannes ^a  

^a Medical University of Vienna   (Austria)

^b MEDICAL UNIVERSITY OF VIENNA   (Austria)

Author keywords

[No Author keywords available]

Indexed keywords

ABC TRANSPORTER; ATP-BINDING CASSETTE TRANSPORTERS; CYTOSOLIC FACTORS; HEAT INACTIVATION; HUMAN FIBROBLAST; LONG-CHAIN ACYL-COA; OXIDATION ACTIVITIES; VERY LONG-CHAIN FATTY ACIDS;

ANTIBODIES; CELL CULTURE; DEFECTS; ESTERIFICATION; ESTERS; FATTY ACIDS; FIBROBLASTS; GENE ENCODING;

OXIDATION;

ABC TRANSPORTER; ABCD1 TRANSPORTER; ABCD3 TRANSPORTER; ACYL COENZYME A; FATTY ACID DERIVATIVE; LONG CHAIN ACYL COENZYME A; LONG CHAIN FATTY ACID; MESSENGER RNA; N ETHYLMALEIMIDE; PROTEIN ANTIBODY; UNCLASSIFIED DRUG;

ADRENOLEUKODYSTROPHY; ANTIBODY SPECIFICITY; ARTICLE; BIOACCUMULATION; BIODEGRADATION; CELLULAR DISTRIBUTION; CONTROLLED STUDY; CYTOSOL; FATTY ACID OXIDATION; FIBROBLAST; HUMAN; HUMAN CELL; HUMAN TISSUE; PEROXISOME; PRIORITY JOURNAL; PROTEIN DEFECT; PROTEIN FUNCTION; X CHROMOSOME LINKED DISORDER; ZELLWEGER SYNDROME;

Β-OXIDATION; ABC TRANSPORTER; ABCD1; ABCD3; ADRENOLEUKODYSTROPHY PROTEIN; FATTY ACID TRANSPORT; METABOLIC DISEASES; PEROXISOMES; VLCFA; X-ALD;

ACYL COENZYME A; ADRENOLEUKODYSTROPHY; ATP-BINDING CASSETTE TRANSPORTERS; BIOLOGICAL TRANSPORT; CYTOSOL; FATTY ACIDS; FIBROBLASTS; HUMANS; MALE; OXYGEN; PEROXISOMES; SKIN; SUBCELLULAR FRACTIONS;

EID: 84879576778     PISSN: 00219258     EISSN: 1083351X     Source Type: Journal    
DOI: 10.1074/jbc.M112.445445     Document Type: Article

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