Metabolism Antibodies 3

Anti-CPS1 Antibody (CAB8080)

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SKU:
CAB8080
Product Type:
Antibody
Applications:
WB
Applications:
IF
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Metabolism

Description

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Antibody Name:CPS1 Rabbit Polyclonal Antibody
Antibody SKU:CAB8080
Antibody Size:20uL, 50uL, 100uL
Application:WB IF
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 820-930 of human CPS1 (NP_001116105.1).
Application:WB IF
Recommended Dilution:WB 1:1000 - 1:2000 IF 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Positive Samples:HepG2, NCI-H460, HeLa, Mouse liver, Rat liver
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 820-930 of human CPS1 (NP_001116105.1).
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:RMCH PSIE GFTP RLPM NKEW PSNL DLRK ELSE PSST RIYA IAKA IDDN MSLD EIEK LTYI DKWF LYKM RDIL NMEK TLKG LNSE SMTE ETLK RAKE IGFS DKQI SKCL GLT
Gene ID:1373
Uniprot:P31327
Cellular Location:Mitochondrion, Nucleus, nucleolus
Calculated MW:116kDa/164kDa/165kDa
Observed MW:165kDa
Synonyms:CPS1, CPSASE1, PHN
Background:The mitochondrial enzyme encoded by this gene catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. This reaction is the first committed step of the urea cycle, which is important in the removal of excess urea from cells. The encoded protein may also represent a core mitochondrial nucleoid protein. Three transcript variants encoding different isoforms have been found for this gene. The shortest isoform may not be localized to the mitochondrion. Mutations in this gene have been associated with carbamoyl phosphate synthetase deficiency, susceptibility to persistent pulmonary hypertension, and susceptibility to venoocclusive disease after bone marrow transplantation.
UniProt Protein Function:CPS1: Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell. Defects in CPS1 are the cause of carbamoyl phosphate synthetase 1 deficiency (CPS1D). CPS1D is an autosomal recessive disorder of the urea cycle causing hyperammonemia. Clinical features include protein intolerance, intermittent ataxia, seizures, lethargy, developmental delay and mental retardation. Genetic variations in CPS1 influence the availability of precursors for nitric oxide (NO) synthesis and play a role in clinical situations where endogenous NO production is critically important, such as neonatal pulmonary hypertension, increased pulmonary artery pressure following surgical repair of congenital heart defects or hepatovenocclusive disease following bone marrow transplantation. Infants with neonatal pulmonary hypertension homozygous for Thr-1406 have lower L-arginine concentrations than neonates homozygous for Asn-1406. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Amino Acid Metabolism - arginine and proline; Nucleolus; Amino Acid Metabolism - alanine, aspartate and glutamate; Ligase; Mitochondrial; Energy Metabolism - nitrogen; EC 6.3.4.16

Chromosomal Location of Human Ortholog: 2q35

Cellular Component: mitochondrial matrix

Molecular Function:aspartate carbamoyltransferase activity; carbamoyl-phosphate synthase (ammonia) activity; carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity; dihydroorotase activity; protein binding

Biological Process: 'de novo' pyrimidine base biosynthetic process; arginine biosynthetic process; glycogen catabolic process; homocysteine metabolic process; nitric oxide metabolic process; positive regulation of vasodilation; response to lipopolysaccharide; triacylglycerol catabolic process; urea cycle

Disease: Carbamoyl Phosphate Synthetase I Deficiency, Hyperammonemia Due To; Pulmonary Hypertension, Neonatal, Susceptibility To

NCBI Summary:The mitochondrial enzyme encoded by this gene catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. This reaction is the first committed step of the urea cycle, which is important in the removal of excess urea from cells. The encoded protein may also represent a core mitochondrial nucleoid protein. Three transcript variants encoding different isoforms have been found for this gene. The shortest isoform may not be localized to the mitochondrion. Mutations in this gene have been associated with carbamoyl phosphate synthetase deficiency, susceptibility to persistent pulmonary hypertension, and susceptibility to venoocclusive disease after bone marrow transplantation.[provided by RefSeq, May 2010]
UniProt Code:P31327
NCBI GenInfo Identifier:4033707
NCBI Gene ID:1373
NCBI Accession:P31327.2
UniProt Secondary Accession:P31327,O43774, Q53TL5, Q59HF8, Q7Z5I5, B7Z818, J3KQL0
UniProt Related Accession:P31327
Molecular Weight:165,650 Da
NCBI Full Name:Carbamoyl-phosphate synthase
NCBI Synonym Full Names:carbamoyl-phosphate synthase 1
NCBI Official Symbol:CPS1  
NCBI Official Synonym Symbols:PHN; CPSASE1  
NCBI Protein Information:carbamoyl-phosphate synthase [ammonia], mitochondrial
UniProt Protein Name:Carbamoyl-phosphate synthase [ammonia], mitochondrial
UniProt Synonym Protein Names:Carbamoyl-phosphate synthetase I; CPSase I
Protein Family:Carboxypeptidase
UniProt Gene Name:CPS1  
UniProt Entry Name:CPSM_HUMAN
Anti-CPS1 Polyclonal Antibody (CAB8080)Western blot analysis of extracts of various cell lines, using CPS1 antibody (CAB8080) 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: 1s.
Anti-CPS1 Polyclonal Antibody (CAB8080)Immunofluorescence analysis of HeLa cells using Anti-CPS1 Polyclonal Antibody (CAB8080) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.
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