X-linked adrenoleukodystrophy (X-ALD) is a severe neurodegenerative disorder biochemically characterized by elevated levels of very long-chain fatty acids (VLCFA). Excess levels of VLCFAs are thought to play an important role in the pathogenesis of X-ALD. Therefore, therapeutic approaches for X-...
5.2Very long chain fatty acids Peroxisomal disordersare characterized by impaired, reduced, or totalabsenceofperoxisomesin cells. These disorders imply an accumulation of verylong chain fatty acids(VLCFA) such as tetracosanoic andhexacosanoic acidsin plasma andred blood cells. Some vari...
将“very long chain fatty acid"自动翻译成 意大利文 acido grasso a catena molto lunga Glosbe Translate acido grasso a catena molto lunga Google Translate 添加示例 在上下文、翻译记忆库中将“very long chain fatty acid"翻译成 意大利文 变形干
Childhood adrenoleukodystrophy (cALD) is a metabolic disorder in which very long-chain fatty acids (VLCFA) accumulate due to ALD protein gene defects, ultimately leading to lipotoxicity-induced neuroinflammatory demyelinating disease. Therefore, we examined VLCFA-mediated alterations in the metabolism of ...
X-linked adrenoleukodystrophy (ALD) is a severe neurodegenerative disorder caused by the accumulation of very long-chain fatty acids (VLCFA) due to mutations in the ABCD1 gene. The phenotypic spectrum ranges from a fatal cerebral demyelinating disease in childhood (cerebral ALD) to a progressive my...
Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) (OMIM #609016) is an autosomal recessively inherited disorder of long-chain fatty acid oxidation with an estimated overall frequency of 1:50,000, first described in 1989 in children presenting with hypoketotic hypoglycemia and lethargy...
Long-chain acyl-CoA dehydrogenase Hale ul. in 1985 ( I ) , at least 13 patients have been reported (LCAD) deficiency is a disorder of fatty acid 8-oxidation. (2.3). The main clinical symptoms of this disease include muscle Its diagnosis has been made based on the reduced activity ...
p>Very long chain acyl-CoA dehydrogenase (VLCAD) deficiency is a life-threatening disorder of mitochondrial fatty acid β-oxidation. We identified four novel mutations in three unrelated patients. All patients had the severe childhood form of VLCAD deficiency with early onset and high mortality. Imm...
Very-long chain acyl-CoA dehydrogenase (VLCAD) deficiency (VLCADD) is an autosomal recessive disorder of fatty acid oxidation. Fatty acids are a major source of energy during catabolic stress, so the absence of VLCAD can result in a metabolic crises and respiratory insufficiency. The etiology of...
Very‐long chain acyl‐CoA dehydrogenase (VLCAD) deficiency (VLCADD) is an autosomal recessive disorder of fatty acid oxidation. Fatty acids are a major source of energy during catabolic stress, so the absence of VLCAD can result in a metabolic crises and respiratory insufficiency. The etiology ...