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Beta-alanine metabolism. Beta-Alanine is the only naturally occurring non-essential beta amino acid. Itis formed in organism by different metabolic pathways. One occurs via reductive pyrimidine degradation and begins with the conversion ofUracil to 5,6-Dihydrouracil by Dihydropyrimidine dehydrogenase (DPYD ) [1], [2]. Then Dihydropyrimidinase ( DPYS )catalyzes the reversible hydrolytic ring opening of dihydrouracil toN-Carbamoyl-beta-alanine [3], which in turn is hydrolyzed tobeta-Alanine by Ureidopropionase, beta ( BUP1 ) [4], [5]. Another main pathway of beta-Alanine biosynthesis is degradation ofbeta-Alanyl-(L)-histidine. Carnosine N-methyltransferase convertsbeta-Alanyl-(L)-histidine to Anserine using S-Adenosyl-L-methionineas methyl donor [6]. Then Anserine is hydrolyzed tobeta-Alanine by Carnosine dipeptidase 1 (metallopeptidase M20 family) CPGL2[7]. Beta-Alanyl-(L)-histidine may also be hydrolyzed by CPGL2to beta-Alanine and (L)-Histidine [8]. (L)-Aspartic acid undergoes decarboxylation to beta-Alanine by Glutamatedecarboxylase 1 (brain, 67kDa) ( GAD1 ) and Glutamate decarboxylase 2 (pancreaticislets and brain, 65kDa) ( GAD2 ) [8] or by Cysteine sulfinic aciddecarboxylase ( CSAD ) [9]. 1, 3-Diaminopropane is involved in the beta-Alanine metabolic pathwayvia formation of 3-Aminopropanal by Amiloride binding protein 1 (amine oxidase(copper-containing)) ( ABP1 ) [10] followed by Aldehyde dehydrogenase 9family, member A1 ( ALD9A1 )-catalyzed oxidation to beta-Alanine [11], [12].




