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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How to move an amphipathic molecule across a lipid bilayer: different mechanisms for different ABC transporters?Peroxisomes in brain development and functionPeroxisomal ABC transporters: functions and mechanismThe genetic landscape of X-linked adrenoleukodystrophy: inheritance, mutations, modifier genes, and diagnosisABC Transporter Subfamily D: Distinct Differences in Behavior between ABCD1-3 and ABCD4 in Subcellular Localization, Function, and Human DiseaseYoung-onset dementiaAbcd2 is a strong modifier of the metabolic impairments in peritoneal macrophages of ABCD1-deficient micePathophysiology of X-linked adrenoleukodystrophy.Autophagy induction halts axonal degeneration in a mouse model of X-adrenoleukodystrophy.Mechanistic insights into PTS2-mediated peroxisomal protein import: the co-receptor PEX5L drastically increases the interaction strength between the cargo protein and the receptor PEX7Evaluation of retinoids for induction of the redundant gene ABCD2 as an alternative treatment option in X-linked adrenoleukodystrophy.Activation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy.An ABCD1 Mutation (c.253dupC) Caused Diverse Phenotypes of Adrenoleukodystrophy in an Iranian Consanguineous Pedigree.ATP-binding cassette transporters in liver.X-linked adrenoleukodystrophy: pathogenesis and treatment.Peroxisome-mitochondria interplay and disease.Peroxisomal Disorders: A Review on Cerebellar Pathologies.Functional characterisation of peroxisomal β-oxidation disorders in fibroblasts using lipidomics.Peroxisomal ATP-binding cassette transporters form mainly tetramers.X-linked adrenoleukodystrophy: very long-chain fatty acid metabolism is severely impaired in monocytes but not in lymphocytes.Adrenoleukodystrophy - neuroendocrine pathogenesis and redefinition of natural history.Clinical and Laboratory Diagnosis of Peroxisomal Disorders.S149R, a novel mutation in the ABCD1 gene causing X-linked adrenoleukodystrophy.Deficiency of a Retinal Dystrophy Protein, Acyl-CoA Binding Domain-containing 5 (ACBD5), Impairs Peroxisomal β-Oxidation of Very-long-chain Fatty Acids.
P2860
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P2860
Impaired very long-chain acyl-CoA β-oxidation in human X-linked adrenoleukodystrophy fibroblasts is a direct consequence of ABCD1 transporter dysfunction.
description
2013 nî lūn-bûn
@nan
2013年の論文
@ja
2013年論文
@yue
2013年論文
@zh-hant
2013年論文
@zh-hk
2013年論文
@zh-mo
2013年論文
@zh-tw
2013年论文
@wuu
2013年论文
@zh
2013年论文
@zh-cn
name
Impaired very long-chain acyl- ...... ABCD1 transporter dysfunction.
@en
type
label
Impaired very long-chain acyl- ...... ABCD1 transporter dysfunction.
@en
prefLabel
Impaired very long-chain acyl- ...... ABCD1 transporter dysfunction.
@en
P2093
P2860
P356
P1476
Impaired very long-chain acyl- ...... ABCD1 transporter dysfunction
@en
P2093
Günther Regelsberger
Markus Kunze
Sonja Forss-Petter
P2860
P304
19269-19279
P356
10.1074/JBC.M112.445445
P407
P577
2013-05-13T00:00:00Z