Excess dietary fatty acids and excess dietary carbohydrate are stored in adipose tissue as triacylglycerols. Insulin is the major hormone that stimulates this process of fat storage. Fatty acids are a prime fuel for humans and lower animals; they can be stored as triacylglycerols in large amounts...
The compounds are suitable, for example, for the treatment and/or prevention of disorders of the fatty acid metabolism and glucose utilization disorders and also disorders in which insulin resistance is involved.Christian StapperStefanie KeilHeiner Glombik...
Two disorders of fatty acid metabolism were described in 1973. Since then, at least 22 different inborn errors of metabolism affecting β-oxidation in skeletal muscle and other tissues have been identified. Neurological findings are prominent in many of these, including hypotonia, myopathy (often ...
Fatty acid oxidation disorders (FAOD) are a group of rare, autosomal recessive, metabolic disorders caused by variants of the genes for the enzymes and proteins involved in the transport and metabolism of fatty acids in the mitochondria. Those affected by FAOD are unable to convert fatty acids ...
Disorders of lipid metabolism The present invention provides isolated or recombinant nucleic acid molecules having one or more mutations linked to disorders of lipid metabolism such as Chylomicron Retention Disease (CMRD), Anderson's Disease (AD), Chylomicron Retenti... CAROL CHRISTINE SHOULDERS,JAMES ...
such as the citric acid cycle, oxidative reactions, gluconeogenesis and the hexose monophosphate shunt.The principles illustrated in this discussion will undoubtedly prove to be applicable to other areas of metabolism as we continue to learn more about the metabolic consequences of acid-base disorders....
Fatty acids are the major metabolic products of lipid metabolism. They are categorised by number of carbon-carbon double bonds: saturated fatty acids have none, monounsaturated fatty acids have one and polyunsaturated fatty acids (PUFAs) have two or more. PUFAs are further subdivided by the posi...
Disorders of fatty acid oxidation and energy metabolism Comparative results were obtained in patients with short, medium, very long-chain and multiple acyl-CoA dehydrogenase deficiencies, and malonyl-CoA decarboxylase deficiency. Several patients with mitochondrial dysfunction were also studied. Urine from ...
Despite these challenges, the approach has already been applied to understand the metabolism in a range of animal models, and has more recently started to be projected into the clinical situation. In this review, the technologies currently being used in metabolomics will be assessed prior to ...
Fatty acid metabolism in cultured skin fi- broblasts from patients with peroxiso- mal disorders: lignoceryl-CoA ligase deficiency in childhood adrenoleuko- dystrophy. Fahimi HD, Seis H, eds. In: Peroxisomes in Medicine and Biology. Springer-Verlag, Heidelberg, 1987; 358-363....