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Adrenoleukodystrophy-related protein can compensate functionally for adrenoleukodystrophy protein deficiency (X-ALD): implications for therapyProgression of abnormalities in adrenomyeloneuropathy and neurologically asymptomatic X-linked adrenoleukodystrophy despite treatment with "Lorenzo's oil"Peroxisomes in brain development and functionAstrocytes are central in the pathomechanisms of vanishing white matterMouse very long-chain acyl-CoA synthetase in X-linked adrenoleukodystrophyABHD12 controls brain lysophosphatidylserine pathways that are deregulated in a murine model of the neurodegenerative disease PHARCFunctional Characterization of IPSC-Derived Brain Cells as a Model for X-Linked AdrenoleukodystrophyC26:0-Carnitine Is a New Biomarker for X-Linked Adrenoleukodystrophy in Mice and ManAbsence of spontaneous peroxisome proliferation in enoyl-CoA Hydratase/L-3-hydroxyacyl-CoA dehydrogenase-deficient mouse liver. Further support for the role of fatty acyl CoA oxidase in PPARalpha ligand metabolismX-linked adrenoleukodystrophy enigma: how does the ALD peroxisomal transporter mutation affect CNS glia?Adrenomyeloneuropathy: a neuropathologic review featuring its noninflammatory myelopathy.Interferons, signal transduction pathways, and the central nervous system.Abcd2 is a strong modifier of the metabolic impairments in peritoneal macrophages of ABCD1-deficient micePEX11 beta deficiency is lethal and impairs neuronal migration but does not abrogate peroxisome functionABCD2 alters peroxisome proliferator-activated receptor α signaling in vitro, but does not impair responses to fenofibrate therapy in a mouse model of diet-induced obesityPathophysiology of X-linked adrenoleukodystrophy.Nonalcoholic steatosis and steatohepatitis. III. Peroxisomal beta-oxidation, PPAR alpha, and steatohepatitis.Liver X receptor alpha interferes with SREBP1c-mediated Abcd2 expression. Novel cross-talk in gene regulation.Pathomechanisms underlying X-adrenoleukodystrophy: a three-hit hypothesis.The mousetrap: what we can learn when the mouse model does not mimic the human disease.Antioxidants halt axonal degeneration in a mouse model of X-adrenoleukodystrophy.Peroxisomal dysfunction in inflammatory childhood white matter disorders: an unexpected contributor to neuropathologyRole of ALDP (ABCD1) and mitochondria in X-linked adrenoleukodystrophy.Histone deacetylase inhibitor upregulates peroxisomal fatty acid oxidation and inhibits apoptotic cell death in abcd1-deficient glial cells.Autophagy induction halts axonal degeneration in a mouse model of X-adrenoleukodystrophy.Oxidative damage compromises energy metabolism in the axonal degeneration mouse model of X-adrenoleukodystrophy.Loss of AMP-activated protein kinase induces mitochondrial dysfunction and proinflammatory response in unstimulated Abcd1-knockout mice mixed glial cells.ABCD1 deletion-induced mitochondrial dysfunction is corrected by SAHA: implication for adrenoleukodystrophy.Mammalian peroxisomal ABC transporters: from endogenous substrates to pathology and clinical significance.The myelin mutants as models to study myelin repair in the leukodystrophies.Deletion of a single allele of the Pex11β gene is sufficient to cause oxidative stress, delayed differentiation and neuronal death in mouse brainFunctional genomic analysis unravels a metabolic-inflammatory interplay in adrenoleukodystrophyPioglitazone halts axonal degeneration in a mouse model of X-linked adrenoleukodystrophyActivation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy.Oxidative stress modulates mitochondrial failure and cyclophilin D function in X-linked adrenoleukodystrophyDietary rescue of altered metabolism gene reveals unexpected Drosophila mating cuesNeurodegeneration in a Drosophila model of adrenoleukodystrophy: the roles of the Bubblegum and Double bubble acyl-CoA synthetases.Plasmalogen deficiency in cerebral adrenoleukodystrophy and its modulation by lovastatinSilencing of Abcd1 and Abcd2 genes sensitizes astrocytes for inflammation: implication for X-adrenoleukodystrophy.Fatty Acid Transport Proteins: Targeting FATP2 as a Gatekeeper Involved in the Transport of Exogenous Fatty Acids.
P2860
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P2860
description
1997 nî lūn-bûn
@nan
1997年の論文
@ja
1997年論文
@yue
1997年論文
@zh-hant
1997年論文
@zh-hk
1997年論文
@zh-mo
1997年論文
@zh-tw
1997年论文
@wuu
1997年论文
@zh
1997年论文
@zh-cn
name
A mouse model for X-linked adrenoleukodystrophy.
@ast
A mouse model for X-linked adrenoleukodystrophy.
@en
type
label
A mouse model for X-linked adrenoleukodystrophy.
@ast
A mouse model for X-linked adrenoleukodystrophy.
@en
prefLabel
A mouse model for X-linked adrenoleukodystrophy.
@ast
A mouse model for X-linked adrenoleukodystrophy.
@en
P2093
P2860
P356
P1476
A mouse model for X-linked adrenoleukodystrophy.
@en
P2093
A M Lawler
J M Powers
P A Watkins
P2860
P304
P356
10.1073/PNAS.94.17.9366
P407
P577
1997-08-01T00:00:00Z