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Pages in category "Amino acid metabolism disorders" ... Template:Amino acid metabolic pathology; ... Branched-chain keto acid dehydrogenase kinase deficiency;
Organic acidemia is a term used to classify a group of metabolic disorders which disrupt normal amino acid metabolism, particularly branched-chain amino acids, causing a buildup of acids which are usually not present. [1] The branched-chain amino acids include isoleucine, leucine and valine. [1]
Amino acid; Amylase (also see α-amylase) Anabolic steroid; Anandamide (ANA) Androgen; Anethole; Angiotensinogen; Anisomycin; Antidiuretic hormone (ADH) Anti-Müllerian hormone (AMH) Arabinose; Arginine; Argonaute; Ascomycin; Ascorbic acid ; Asparagine; Aspartic acid; Asymmetric dimethylarginine; ATP synthase; Atrial-natriuretic peptide (ANP ...
Traditionally the inherited metabolic diseases were classified as disorders of carbohydrate metabolism, amino acid metabolism, organic acid metabolism, or lysosomal storage diseases. [4] In recent decades, hundreds of new inherited disorders of metabolism have been discovered and the categories have proliferated.
The essential amino acids valine, methionine, isoleucine, and threonine can not be converted and this leads to a buildup of propionyl-CoA. Instead of being converted to methylmalonyl-CoA, propionyl-CoA is then converted into propionic acid, which builds up in the bloodstream. This in turn causes an accumulation of dangerous acids and toxins ...
Niacin deficiency is a consequence of a diet low in both niacin and the amino acid tryptophan, a precursor for the vitamin. Low plasma tryptophan is a non-specific indicator, meaning it can have other causes. The signs and symptoms of niacin deficiency start to revert within days of oral supplementation with large amounts of the vitamin. [23] [24]
Microorganisms and plants synthesize many uncommon amino acids. For example, some microbes make 2-aminoisobutyric acid and lanthionine, which is a sulfide-bridged derivative of alanine. Both of these amino acids are found in peptidic lantibiotics such as alamethicin. [116]
The absorption rates of individual amino acids are highly dependent on the protein source; for example, the digestibilities of many amino acids in humans, the difference between soy and milk proteins [32] and between individual milk proteins, beta-lactoglobulin and casein. [33]