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Anti-UPB1 Antibody (CAB15452)

SKU:
CAB15452
Product Type:
Antibody
Reactivity:
Mouse
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cell Biology
€139 - €419
Frequently bought together:

Description

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Antibody Name:Anti-UPB1 Antibody
Antibody SKU:CAB15452
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-240 of human UPB1 (NP_057411.1).
Application:WB
Recommended Dilution:WB 1:200 - 1:2000
Reactivity:Mouse, Rat
Positive Samples:Mouse kidney, Mouse liver, Rat kidney
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-240 of human UPB1 (NP_057411.1).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence: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
Gene ID:51733
Uniprot:Q9UBR1
Cellular Location:Cytoplasm
Calculated MW:43kDa
Observed MW:43kDa
Synonyms:UPB1, BUP1
Background: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
Anti-UPB1 Antibody (CAB15452)Western blot analysis of extracts of various cell lines, using UPB1 antibody (CAB15452) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 30s.

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