Activation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy
نویسندگان
چکیده
منابع مشابه
assessment of the effect of honey as a topical therapy for intra oral wound healing in rat.
چکیده ندارد.
15 صفحه اولPeroxisomal β-oxidation in X-linked adrenoleukodystrophy
Background: ABCD1 is a peroxisomal ABC transporter whose dysfunction causes X-linked adrenoleukodystrophy (X-ALD). Results: β-Oxidation of C26:0 as well as C22:0 acyl-CoA esters is impaired in X-ALD. ABCD3 accounts for residual β-oxidation activity in XALD fibroblasts. Conclusion: ABCD1 mediates very long-chain acyl-CoA ester β-oxidation without need for additional re-esterification by an acyl-...
متن کاملnano-rods zno as an efficient catalyst for the synthesis of chromene phosphonates, direct amidation and formylation of amines
چکیده ندارد.
Impaired mitochondrial oxidative phosphorylation in the peroxisomal disease X-linked adrenoleukodystrophy.
X-linked adrenoleukodystrophy (X-ALD) is an inherited metabolic disorder of the nervous system characterized by axonopathy in spinal cords and/or cerebral demyelination, adrenal insufficiency and accumulation of very long-chain fatty acids (VLCFAs) in plasma and tissues. The disease is caused by malfunction of the ABCD1 gene, which encodes a peroxisomal transporter of VLCFAs or VLCFA-CoA. In th...
متن کاملX-linked adrenoleukodystrophy presenting as Addison disease.
skin, starting from face since the age of 4 years. There was history of recurrent episodes of loose motions, vomiting and fever after the age of 4 years in the elder sibling for which he was hospitalized in a state of shock. Family history, perinatal history and developmental history were non-contributory. General and systemic examinations were unremarkable except for the generalized hyperpigme...
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ژورنال
عنوان ژورنال: Cell Death & Differentiation
سال: 2015
ISSN: 1350-9047,1476-5403
DOI: 10.1038/cdd.2015.20