Gene Association: ALDH6A1
UniProt Search:
ALDH6A1 (PROTEIN_CODING)
Function Description: aldehyde dehydrogenase 6 family member A1
found 9 associated metabolites with current gene based on the text mining result from the pubmed database.
temephos
D010575 - Pesticides > D007306 - Insecticides D016573 - Agrochemicals
Beta-Alanine
beta-Alanine is the only naturally occurring beta-amino acid - an amino acid in which the amino group is at the beta-position from the carboxylate group. It is formed in vivo by the degradation of dihydrouracil and carnosine. It is a component of the naturally occurring peptides carnosine and anserine and also of pantothenic acid (vitamin B-5), which itself is a component of coenzyme A. Under normal conditions, beta-alanine is metabolized into acetic acid. beta-Alanine can undergo a transanimation reaction with pyruvate to form malonate-semialdehyde and L-alanine. The malonate semialdehyde can then be converted into malonate via malonate-semialdehyde dehydrogenase. Malonate is then converted into malonyl-CoA and enter fatty acid biosynthesis. Since neuronal uptake and neuronal receptor sensitivity to beta-alanine have been demonstrated, beta-alanine may act as a false transmitter replacing gamma-aminobutyric acid. When present in sufficiently high levels, beta-alanine can act as a neurotoxin, a mitochondrial toxin, and a metabotoxin. A neurotoxin is a compound that damages the brain or nerve tissue. A mitochondrial toxin is a compound that damages mitochondria and reduces cellular respiration as well as oxidative phosphorylation. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of beta-alanine are associated with at least three inborn errors of metabolism, including GABA-transaminase deficiency, hyper-beta-alaninemia, and methylmalonate semialdehyde dehydrogenase deficiency. beta-Alanine is a central nervous system (CNS) depressant and is an inhibitor of GABA transaminase. The associated inhibition of GABA transaminase and displacement of GABA from CNS binding sites can also lead to GABAuria (high levels of GABA in the urine) and convulsions. In addition to its neurotoxicity, beta-alanine reduces cellular levels of taurine, which are required for normal respiratory chain function. Cellular taurine depletion is known to reduce respiratory function and elevate mitochondrial superoxide generation, which damages mitochondria and increases oxidative stress (PMID: 27023909). Individuals suffering from mitochondrial defects or mitochondrial toxicity typically develop neurotoxicity, hypotonia, respiratory distress, and cardiac failure. beta-Alanine is a biomarker for the consumption of meat, especially red meat. Widely distributed in plants including algae, fungi and many higher plants. Flavouring ingredient β-Alanine. CAS Common Chemistry. CAS, a division of the American Chemical Society, n.d. https://commonchemistry.cas.org/detail?cas_rn=107-95-9 (retrieved 2024-07-01) (CAS RN: 107-95-9). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0). β-Alanine is a non-essential amino acid that is shown to be metabolized into carnosine, which functions as an intracellular buffer. β-Alanine is a non-essential amino acid that is shown to be metabolized into carnosine, which functions as an intracellular buffer. β-Alanine is a non-essential amino acid that is shown to be metabolized into carnosine, which functions as an intracellular buffer.
2-Methylcitric acid
Methylcitric acid (MCA) is elevated in body fluids of patients with propionic acidaemia (PA; OMIM 232000, 232050), methylmalonic aciduria (MMA; OMIM 251000, 251120) and multiple carboxylase deficiency (OMIM 253260, 253270), which are inherited disorders. MCA is formed by condensation of accumulated propionyl- CoA and oxalacetate by the enzyme si-citrate synthase (EC 4.1.3.7). MCA molecule has two stereogenic centers so that it can occur in the form of four stereoisomers. Only two stereoisomers of MCA, (2S, 3S) and (2R, 3S), were found in human urine (PMID: 17295121). Methylcitric acid (MCA) is elevated in body fluids of patients with propionic acidaemia (PA; OMIM 232000, 232050), methylmalonic aciduria (MMA; OMIM 251000, 251120) and multiple carboxylase deficiency (OMIM 253260, 253270). MCA is formed by condensation of accumulated propionyl- CoA and oxalacetate by the enzyme si-citrate synthase (EC 4.1.3.7). MCA molecule has two stereogenic centers so that it can occur in the form of four stereoisomers. Only two stereoisomers of MCA, (2S, 3S) and (2R, 3S), were found in human urine. (PMID: 17295121) [HMDB] 2-Methylcitric acid (Methylcitric acid) is an endogenous metabolite in the 2-methylcitric acid cycle. 2-Methylcitric acid accumulates in methylmalonic and propionic acidemias and acts as a marker metabolite. 2-Methylcitric acid markedly inhibits ADP-stimulated and uncoupled respiration in mitochondria supported by glutamate[1]. 2-Methylcitric acid (Methylcitric acid) is an endogenous metabolite in the 2-methylcitric acid cycle. 2-Methylcitric acid accumulates in methylmalonic and propionic acidemias and acts as a marker metabolite. 2-Methylcitric acid markedly inhibits ADP-stimulated and uncoupled respiration in mitochondria supported by glutamate[1]. 2-Methylcitric acid (Methylcitric acid) is an endogenous metabolite in the 2-methylcitric acid cycle. 2-Methylcitric acid accumulates in methylmalonic and propionic acidemias and acts as a marker metabolite. 2-Methylcitric acid markedly inhibits ADP-stimulated and uncoupled respiration in mitochondria supported by glutamate[1].
2-Methyl-3-oxopropanoic acid
2-Methyl-3-oxopropanoic acid is an intermediate in the metabolism of Propanoate. It is a substrate for 3-hydroxyisobutyrate dehydrogenase (mitochondrial), Alanine--glyoxylate aminotransferase 2 (mitochondrial) and Methylmalonate-semialdehyde dehydrogenase (mitochondrial). [HMDB] 2-Methyl-3-oxopropanoic acid is an intermediate in the metabolism of Propanoate. It is a substrate for 3-hydroxyisobutyrate dehydrogenase (mitochondrial), Alanine--glyoxylate aminotransferase 2 (mitochondrial) and Methylmalonate-semialdehyde dehydrogenase (mitochondrial).
3-Hydroxyisobutyric acid
A 4-carbon, branched hydroxy fatty acid and intermediate in the metabolism of valine. 3-Hydroxyisobutyric acid is an important interorgan metabolite, an intermediate in the pathways of l-valine and thymine and a good gluconeogenic substrate.
β-alanine
A naturally-occurring beta-amino acid comprising propionic acid with the amino group in the 3-position. β-Alanine is a non-essential amino acid that is shown to be metabolized into carnosine, which functions as an intracellular buffer. β-Alanine is a non-essential amino acid that is shown to be metabolized into carnosine, which functions as an intracellular buffer. β-Alanine is a non-essential amino acid that is shown to be metabolized into carnosine, which functions as an intracellular buffer.
temephos
D010575 - Pesticides > D007306 - Insecticides D016573 - Agrochemicals
2-methyl-3-oxopropanoic acid
The 2-methyl-3-oxo derivative of propanoic acid.
2-Methylcitric acid
2-Methylcitric acid (Methylcitric acid) is an endogenous metabolite in the 2-methylcitric acid cycle. 2-Methylcitric acid accumulates in methylmalonic and propionic acidemias and acts as a marker metabolite. 2-Methylcitric acid markedly inhibits ADP-stimulated and uncoupled respiration in mitochondria supported by glutamate[1]. 2-Methylcitric acid (Methylcitric acid) is an endogenous metabolite in the 2-methylcitric acid cycle. 2-Methylcitric acid accumulates in methylmalonic and propionic acidemias and acts as a marker metabolite. 2-Methylcitric acid markedly inhibits ADP-stimulated and uncoupled respiration in mitochondria supported by glutamate[1]. 2-Methylcitric acid (Methylcitric acid) is an endogenous metabolite in the 2-methylcitric acid cycle. 2-Methylcitric acid accumulates in methylmalonic and propionic acidemias and acts as a marker metabolite. 2-Methylcitric acid markedly inhibits ADP-stimulated and uncoupled respiration in mitochondria supported by glutamate[1].
