Impaired Very Long-chain Acyl-CoA β-Oxidation in Human X-linked Adrenoleukodystrophy Fibroblasts Is a Direct Consequence of ABCD1 Transporter Dysfunction
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Title
Impaired Very Long-chain Acyl-CoA β-Oxidation in Human X-linked Adrenoleukodystrophy Fibroblasts Is a Direct Consequence of ABCD1 Transporter Dysfunction
Authors
Keywords
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Journal
JOURNAL OF BIOLOGICAL CHEMISTRY
Volume 288, Issue 26, Pages 19269-19279
Publisher
American Society for Biochemistry & Molecular Biology (ASBMB)
Online
2013-05-14
DOI
10.1074/jbc.m112.445445
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- ABC Transporters and Immunity: Mechanism of Self-Defense
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- Peroxisomal ABC transporters: Structure, function and role in disease
- (2012) Masashi Morita et al. BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
- X-linked adrenoleukodystrophy: Clinical, metabolic, genetic and pathophysiological aspects
- (2012) Stephan Kemp et al. BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
- Peroxisomal Fatty Acid Uptake Mechanism inSaccharomyces cerevisiae
- (2012) Carlo W. T. van Roermund et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Mutations in ABCD4 cause a new inborn error of vitamin B12 metabolism
- (2012) David Coelho et al. NATURE GENETICS
- The human geneSLC25A17encodes a peroxisomal transporter of coenzyme A, FAD and NAD+
- (2011) Gennaro Agrimi et al. BIOCHEMICAL JOURNAL
- Transfer of metabolites across the peroxisomal membrane
- (2011) Vasily D. Antonenkov et al. BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
- Structural Requirements for Interaction of Peroxisomal Targeting Signal 2 and Its Receptor PEX7
- (2011) Markus Kunze et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Differential substrate specificities of human ABCD1 and ABCD2 in peroxisomal fatty acid β-oxidation
- (2010) Carlo W.T. van Roermund et al. BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS
- General Aspects and Neuropathology of X-Linked Adrenoleukodystrophy
- (2010) Isidro Ferrer et al. BRAIN PATHOLOGY
- Current and Future Pharmacological Treatment Strategies in X-Linked Adrenoleukodystrophy
- (2010) Johannes Berger et al. BRAIN PATHOLOGY
- The role of ELOVL1 in very long-chain fatty acid homeostasis and X-linked adrenoleukodystrophy
- (2010) Rob Ofman et al. EMBO Molecular Medicine
- 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
- (2008) Yoshinori Kashiwayama et al. EXPERIMENTAL CELL RESEARCH
- The human peroxisomal ABC half transporter ALDP functions as a homodimer and accepts acyl-CoA esters
- (2008) Carlo W. T. van Roermund et al. FASEB JOURNAL
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