Anti-UPB1 Antibody (CAB15452)
- SKU:
- CAB15452
- Product type:
- Antibody
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
抗体名: | Anti-UPB1 Antibody |
抗体コード: | CAB15452 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB |
反応性: | Mouse, Rat |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-240 of human UPB1 (NP_057411.1). |
申し込み: | WB |
推奨希釈: | WB 1:200 - 1:2000 |
反応性: | Mouse, Rat |
ポジティブサンプル: | Mouse kidney, Mouse liver, Rat kidney |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-240 of human UPB1 (NP_057411.1). |
精製方法: | Affinity purification |
ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
アイソタイプ: | IgG |
順序: | MAGA EWKS LEEC LEKH LPLP DLQE VKRV LYGK ELRK LDLP REAF EAAS REDF ELQG YAFE AAEE QLRR PRIV HVGL VQNR IPLP ANAP VAEQ VSAL HRRI KAIV EVAA MCGV NIIC FQEA WTMP FAFC TREK LPWT EFAE SAED GPTT RFCQ KLAK NHDM VVVS PILE RDSE HGDV LWNT AVVI SNSG AVLG KTRK NHIP RVGD FNES TYYM EGNL GHPV FQTQ FGRI AVNI CYGR HHPL |
遺伝子ID: | 51733 |
Uniprot: | Q9UBR1 |
セルラーロケーション: | Cytoplasm |
計算された分子量: | 43kDa |
観察された分子量: | 43kDa |
同義語: | UPB1, BUP1 |
バックグラウンド: | 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. |
UniProt Protein Function: | UPB1: Converts N-carbamyl-beta-aminoisobutyric acid and N- carbamyl-beta-alanine to, respectively, beta-aminoisobutyric acid and beta-alanine, ammonia and carbon dioxide. Defects in UPB1 are the cause of beta-ureidopropionase deficiency (BUPD). It is characterized by muscular hypotonia, dystonic movements, scoliosis, microcephaly and severe developmental delay. Patients show strongly elevated levels of N- carbamyl-beta-alanine and N-carbamyl-beta-aminoisobutyric acid in plasma, cerebrospinal fluid and urine. Belongs to the CN hydrolase family. BUP subfamily. |
UniProt Protein Details: | Protein type:Other Amino Acids Metabolism - beta-alanine; Nucleotide Metabolism - pyrimidine; Hydrolase; Xenobiotic Metabolism - drug metabolism - other enzymes; Cofactor and Vitamin Metabolism - pantothenate and CoA biosynthesis; EC 3.5.1.6 Chromosomal Location of Human Ortholog: 22q11.2 Cellular Component: cytosol Molecular Function:beta-ureidopropionase activity Biological Process: pyrimidine nucleoside catabolic process Disease: Beta-ureidopropionase Deficiency |
NCBI Summary: | 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] |
UniProt Code: | Q9UBR1 |
NCBI GenInfo Identifier: | 126153364 |
NCBI Gene ID: | 51733 |
NCBI Accession: | BC131703 |
UniProt Secondary Accession: | Q9UBR1,Q9UIR3, A3KMF8, |
UniProt Related Accession: | Q9UBR1 |
Molecular Weight: | 43,166 Da |
NCBI Full Name: | Homo sapiens ureidopropionase, beta, mRNA |
NCBI Synonym Full Names: | beta-ureidopropionase 1 |
NCBI Official Symbol: | UPB1 |
NCBI Official Synonym Symbols: | BUP1 |
NCBI Protein Information: | beta-ureidopropionase |
UniProt Protein Name: | Beta-ureidopropionase |
UniProt Synonym Protein Names: | BUP-1; Beta-alanine synthase; N-carbamoyl-beta-alanine amidohydrolase |
Protein Family: | Transcription factor |
UniProt Gene Name: | UPB1 |
UniProt Entry Name: | BUP1_HUMAN |
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