Rabbit anti-Human CPS1 Polyclonal Antibody | anti-CPS1 antibody

CPS1, NT (CPS1, Carbamoyl-phosphate synthase [ammonia], mitochondrial, Carbamoyl-phosphate synthetase I)

Cat. Num. AAA647457

• Gene Names: CPS1; PHN; CPSASE1
• Reactivity: Human
• Applications: ELISA, Western Blot
• Purity: Affinity Purified; Purified by Protein A affinity chromatography.
• Synonyms: CPS1; Polyclonal Antibody; NT (CPS1; Carbamoyl-phosphate synthase [ammonia]; mitochondrial; Carbamoyl-phosphate synthetase I); Anti -CPS1; anti-CPS1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Human
• Purity/Purification: Affinity Purified; Purified by Protein A affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

• Applicable Applications for anti-CPS1 antibody: ELISA (EL/EIA), Western Blot (WB)
• Application Notes: Suitable for use in Western Blot, ELISA; Dilution: ELISA: 1:1,000; Western Blot: 1:100-500
• Immunogen: CPS1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 261-291 amino acids from the N-terminal region of human CPS1.
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(CPS1 Antibody (N-term) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the CPS1 antibody detected the CPS1 protein (arrow).)

Related Product Information for anti-CPS1 antibody

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.

Product Categories/Family for anti-CPS1 antibody

Antibodies; Abs to Mitochondrial Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 170295797
• NCBI GeneID: 1373
• NCBI Accession #: NP_001116106.1
• NCBI GenBank Nucleotide #: NM_001122634.2
• UniProt Accession #: P31327; O43774; Q53TL5; Q59HF8; Q7Z5I5; B7Z818; J3KQL0
• Molecular Weight: 164,939 Da
• NCBI Official Full Name: carbamoyl-phosphate synthase
• NCBI Official Synonym Full Names: carbamoyl-phosphate synthase 1, mitochondrial
• NCBI Official Symbol: CPS1
• NCBI Official Synonym Symbols: PHN; CPSASE1
• NCBI Protein Information: carbamoyl-phosphate synthase [ammonia], mitochondrial; carbamoyl-phosphate synthase [ammonia], mitochondrial; carbamoylphosphate synthetase I
• UniProt Protein Name: Carbamoyl-phosphate synthase [ammonia], mitochondrial
• Protein Family: Carboxypeptidase
• UniProt Gene Name: CPS1
• UniProt Synonym Gene Names: CPSase I
• UniProt Entry Name: CPSM_HUMAN

NCBI Description

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 Description

Function: Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell. HAMAP-Rule MF_01209

Catalytic activity: 2 ATP + NH3 + CO2 + H2O = 2 ADP + phosphate + carbamoyl phosphate. HAMAP-Rule MF_01209

Enzyme regulation: Requires N-acetyl-L-glutamate (NAG) as an allosteric activator. HAMAP-Rule MF_01209

Subcellular location: Mitochondrion. Nucleus › nucleolus Ref.15.

Tissue specificity: Primarily in the liver and small intestine.

Domain: The type-1 glutamine amidotransferase domain is defective. HAMAP-Rule MF_01209

Post-translational modification: Succinylated at Lys-287 and Lys-1291. Desuccinylated at Lys-1291 by SIRT5, leading to activation

By similarity. HAMAP-Rule MF_01209

Involvement in disease: Carbamoyl phosphate synthetase 1 deficiency (CPS1D) [MIM:237300]: An autosomal recessive disorder of the urea cycle causing hyperammonemia. It can present as a devastating metabolic disease dominated by severe hyperammonemia in neonates or as a more insidious late-onset condition, generally manifesting as life-threatening hyperammonemic crises under catabolic situations. Clinical features include protein intolerance, intermittent ataxia, seizures, lethargy, developmental delay and mental retardation.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.2 Ref.5 Ref.6 Ref.17 Ref.18 Ref.20 Ref.21 Ref.22 Ref.23 Ref.24 Ref.25 Ref.26Genetic 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. Ref.25

Sequence similarities: Contains 2 ATP-grasp domains.Contains 1 glutamine amidotransferase type-1 domain.

Sequence caution: The sequence BAD92037.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.