General (medium-chain) acyl-CoA dehydrogenase deficiency (non-ketotic dicarboxylic aciduria): quantitative urinary excretion pattern of 23 biologically significant organic acids in three cases
References (27)
- et al.
In vitro fibroblast studies in a patient with C6–C10-dicarboxylic aciduria: evidence for a defect in general acyl-CoA dehydrogenase
Clin Chim Acta
(1982) - et al.
Suberylglycine excretion in the urine from a patient with dicarboxylic aciduria
Clin Chim Acta
(1976) - et al.
Non-ketotic C6–C10-dicarboxylic aciduria: biochemical investigation of two cases
Clin Chim Acta
(1980) - et al.
Dicarboxylic aciduria: the response to fasting
Clin Chim Acta
(1979) - et al.
The biological origin of ketotic dicarboxylic aciduria. II. In vivo and in vitro investigations of the β-oxidation of C8–C16-dicarboxylic acids in unstarved, starved and diabetic rats
Biochim Biophys Acta
(1982) - et al.
Cyanide insensitive and clofibrate enhanced β-oxidation of dodecanedioic acid in rat liver: evidence of peroxisomal β-oxidation of N-dicarboxylic acids
Biochim Biophys Acta
(1982) On the mode of action of hypoglycin A
J Biol Chem
(1972)- et al.
The occurrence of adipic and suberic acid in urine from ketotic patients
Clin Chim Acta
(1972) Rat liver peroxisomes catalyze the β-oxidation of fatty acids
J Biol Chem
(1978)- et al.
Acyl-CoA oxidase of rat liver: a new enzyme for fatty acid oxidation
Biochem Biophys Res Comm
(1978)
Fatty acid oxidation by human liver peroxisomes
Biochem Biophys Res Comm
Glutaric aciduria type II: report on a previously undescribed metabolic disorder
Clin Chim Acta
Glutaric aciduria type II
J Pediat
Cited by (68)
Heptanoic and medium branched-chain fatty acids as anaplerotic treatment for medium chain acyl-CoA dehydrogenase deficiency
2023, Molecular Genetics and MetabolismNeonatal Metabolic Crises: A Practical Approach
2020, Clinics in PerinatologyCitation Excerpt :Biochemical testing typically shows elevations of medium-length acylcarnitines (C6–C10) with prominent octanoylcarnitine (C8) on acylcarnitine profile analysis.13 Urine organic acids show a characteristic pattern of elevated medium-length dicarboxylic acids (C6>C8>C10) with inappropriately low ketones.14 During an acute metabolic event, urine acylglycines will show elevations in hexanoylglycine, suberylglycine, and at times propionylglycine.11
Oxidized phosphatidylcholines suggest oxidative stress in patients with medium-chain acyl-CoA dehydrogenase deficiency
2015, TalantaCitation Excerpt :This is achieved by increasing feeding frequency with food containing a high starch content and a limited supply of medium chain triglyceride oils. Acute patients exhibit hyperamonemia and characteristic profile of elevated organic acids in urine – adipic, suberic, sebacic acids, hexanoic, octanoic acids [11] – and several other glycine conjugates – hexanoylglycine, 3-phenylpropionylglycine and suberylglycine [12]. This biochemical picture depends on the clinical status and can disappear during the period of normalcy.
ω-oxidation of α-chlorinated fatty acids: Identification of α-chlorinated dicarboxylic acids
2010, Journal of Biological ChemistryCitation Excerpt :Thus, ω-oxidation may serve as the primary metabolic pathway for α-ClFA. The utilization of this pathway for α-ClFA catabolism would be analogous to the use of this pathway in children with in-born errors of β-oxidation of fatty acids, which leads to elevated urinary AdA levels (45, 51). Thus, either deficiencies in normal oxidation of fatty acids through genetic deficiencies or poor substrate use for β-oxidation may lead to ω-oxidation and subsequent α-dicarboxylic acid production.
Biosynthesis, degradation and pharmacological importance of the fatty acid amides
2008, Drug Discovery Today