基因名称(Gene Name)
UPB1;BUP1
蛋白名称
Beta-ureidopropionase;BUP-1;Beta-alanine synthase;N-carbamoyl-beta-alanine amidohydrolase;
组成
PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
来源
Monoclonal, Rabbit,IgG
稀释比例
WB 1:1000-5000; IHC 1:100-300; IP 1:50-100 Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0
储存
-15°C to -25°C/1 year(Do not lower than -25°C)
其他名称
UPB1 ; BUP1 ; Beta-ureidopropionase ; BUP-1 ; Beta-alanine synthase ; N-carbamoyl-beta-alanine amidohydrolase ;
背景
This gene encodes a protein that belongs to the CN hydrolase family. Beta-ureidopropionase catalyzes the last step in the pyrimidine degradation pathway. The pyrimidine bases uracil and thymine are degraded via the consecutive action of dihydropyrimidine dehydrogenase (DHPDH), dihydropyrimidinase (DHP) and beta-ureidopropionase (UP) to beta-alanine and beta-aminoisobutyric acid, respectively. UP deficiencies are associated with N-carbamyl-beta-amino aciduria and may lead to abnormalities in neurological activity. [provided by RefSeq, Jul 2008]
功能
Catalyzes a late step in pyrimidine degradation (PubMed:22525402, PubMed:24526388). Converts N-carbamoyl-beta-alanine (3-ureidopropanoate) into beta-alanine, ammonia and carbon dioxide (PubMed:10415095, PubMed:10542323, PubMed:11508704, PubMed:22525402, PubMed:24526388, PubMed:29976570). Likewise, converts N-carbamoyl-beta-aminoisobutyrate (3-ureidoisobutyrate) into beta-aminoisobutyrate, ammonia and carbon dioxide (Probable). {ECO:0000269|PubMed:10415095, ECO:0000269|PubMed:10542323, ECO:0000269|PubMed:11508704, ECO:0000269|PubMed:22525402, ECO:0000269|PubMed:24526388, ECO:0000269|PubMed:29976570, ECO:0000305|PubMed:22525402, ECO:0000305|PubMed:24526388}.
