Lignoceric acid is oxidized in the peroxisome: implications for the Zellweger cerebro-hepato-renal syndrome and adrenoleukodystrophy.
about
Very long-chain acyl-CoA synthetases. Human "bubblegum" represents a new family of proteins capable of activating very long-chain fatty acidsHuman peroxisomal 3-oxoacyl-coenzyme A thiolase deficiencyGenetic heterogeneity in the cerebrohepatorenal (Zellweger) syndrome and other inherited disorders with a generalized impairment of peroxisomal functions. A study using complementation analysisLarge deletion of the peroxisomal acyl-CoA oxidase gene in pseudoneonatal adrenoleukodystrophyAltered expression of ALDP in X-linked adrenoleukodystrophyProgression of abnormalities in adrenomyeloneuropathy and neurologically asymptomatic X-linked adrenoleukodystrophy despite treatment with "Lorenzo's oil"Fatty acid metabolism and the basis of brown adipose tissue functionAdrenoleukodystrophy: molecular genetics, pathology, and Lorenzo's oilMouse very long-chain acyl-CoA synthetase in X-linked adrenoleukodystrophyFunctional Characterization of IPSC-Derived Brain Cells as a Model for X-Linked AdrenoleukodystrophyMolecular cloning of cDNA encoding rat very long-chain acyl-CoA synthetaseAdrenoleukodystrophy and related disorders.Therapy of X-linked adrenoleukodystrophyAdrenoleukodystrophy in a mother and son.Peroxisomal lignoceroyl-CoA ligase deficiency in childhood adrenoleukodystrophy and adrenomyeloneuropathy.A missense point mutation (Ser515Phe) in the adrenoleukodystrophy gene in a family with adrenomyeloneuropathy: a clinical, biochemical, and genetic study.Biology of senescent liver peroxisomes: role in hepatocellular aging and diseaseImpaired degradation of leukotrienes in patients with peroxisome deficiency disorders.Retinal very long-chain PUFAs: new insights from studies on ELOVL4 proteinMyelin and disorders that affect the formation and maintenance of this sheath.Hydroxyeicosatetraenoic acid metabolism in cultured human skin fibroblasts. Evidence for peroxisomal beta-oxidationPlasma very long chain fatty acids in 3,000 peroxisome disease patients and 29,000 controls.Peroxisomal bifunctional enzyme deficiencyPeroxisomal beta-oxidation enzyme proteins in adrenoleukodystrophy: distinction between X-linked adrenoleukodystrophy and neonatal adrenoleukodystrophy.Retroviral-mediated gene transfer corrects very-long-chain fatty acid metabolism in adrenoleukodystrophy fibroblasts.Histone deacetylase inhibitor upregulates peroxisomal fatty acid oxidation and inhibits apoptotic cell death in abcd1-deficient glial cells.A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase (pseudo-neonatal adrenoleukodystrophy)HDAC inhibitor SAHA normalizes the levels of VLCFAs in human skin fibroblasts from X-ALD patients and downregulates the expression of proinflammatory cytokines in Abcd1/2-silenced mouse astrocytes.ABCD1 deletion-induced mitochondrial dysfunction is corrected by SAHA: implication for adrenoleukodystrophy.Impaired mitochondrial fat oxidation induces adaptive remodeling of muscle metabolism.Novel subtype of peroxisomal acyl-CoA oxidase deficiency and bifunctional enzyme deficiency with detectable enzyme protein: identification by means of complementation analysis.Lipid-lowering drugs.Ultrastructural and cytochemical demonstration of peroxisomes in cultured fibroblasts from patients with peroxisomal deficiency disorders.X linked adrenoleukodystrophy: clinical presentation, diagnosis, and therapy.Caffeic acid phenethyl ester induces adrenoleukodystrophy (Abcd2) gene in human X-ALD fibroblasts and inhibits the proinflammatory response in Abcd1/2 silenced mouse primary astrocytes.25-hydroxycholesterol contributes to cerebral inflammation of X-linked adrenoleukodystrophy through activation of the NLRP3 inflammasome.Biosynthesis and maturation of peroxisomal beta-oxidation enzymes in fibroblasts in relation to the Zellweger syndrome and infantile Refsum diseasePeroxisomes: a nexus for lipid metabolism and cellular signaling.Peroxisomal disorders. A review of a recently recognized group of clinical entities.Peroxisome-mitochondria interplay and disease.
P2860
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P2860
Lignoceric acid is oxidized in the peroxisome: implications for the Zellweger cerebro-hepato-renal syndrome and adrenoleukodystrophy.
description
1984 nî lūn-bûn
@nan
1984年の論文
@ja
1984年学术文章
@wuu
1984年学术文章
@zh-cn
1984年学术文章
@zh-hans
1984年学术文章
@zh-my
1984年学术文章
@zh-sg
1984年學術文章
@yue
1984年學術文章
@zh
1984年學術文章
@zh-hant
name
Lignoceric acid is oxidized in ...... rome and adrenoleukodystrophy.
@ast
Lignoceric acid is oxidized in ...... rome and adrenoleukodystrophy.
@en
type
label
Lignoceric acid is oxidized in ...... rome and adrenoleukodystrophy.
@ast
Lignoceric acid is oxidized in ...... rome and adrenoleukodystrophy.
@en
prefLabel
Lignoceric acid is oxidized in ...... rome and adrenoleukodystrophy.
@ast
Lignoceric acid is oxidized in ...... rome and adrenoleukodystrophy.
@en
P2093
P2860
P356
P1476
Lignoceric acid is oxidized in ...... rome and adrenoleukodystrophy.
@en
P2093
P2860
P304
P356
10.1073/PNAS.81.13.4203
P407
P577
1984-07-01T00:00:00Z