Carbamoyl-phosphate synthase [ammonia], mitochondrial; Carbamoyl-phosphate synthetase I; CPSase I

For Research Use Only. Not for use in diagnostic procedures.

Natural and recombinant Mouse Carbamoyl-phosphate synthase [ammonia], mitochondrial

For long term storage, please store the entire kit at -20 degree C.

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Small volumes of CPS1 elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS2890298 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the CPS1/Carbamoyl-Phosphate Synthase [Ammonia], Mitochondrial, ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing CPS1. The ELISA analytical biochemical technique of the MBS2890298 kit is based on CPS1 antibody-CPS1 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect CPS1 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, CPS1. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

carbamoyl-phosphate synthetase 1

carbamoyl-phosphate synthase [ammonia], mitochondrial

Carbamoyl-phosphate synthase [ammonia], mitochondrial

CPSase I??[Similar Products]

CPSM_MOUSE

This gene encodes a protein localized to the inner mitochondrial matrix. The encoded protein plays a role in the detoxification of ammonia by catalyzing the first step in the urea cycle in which carbomyl-phosphate is synthesized from ammonia and bicarbonate. Carbamoyl-phosphate is subsequently converted to urea that is excreted by the kidneys. Deficiency of the encoded enzyme leads to an accumulation of ammonia in the blood. High levels of ammonia are toxic to the central nervous system and result in neurological disorders. [provided by RefSeq, Oct 2013]

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.

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

Cellular Component: protein complex; mitochondrion; cytoplasm; mitochondrial inner membrane; nucleus

Molecular Function: carbamoyl-phosphate synthase (ammonia) activity; protein binding; glutamate binding; carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity; metal ion binding; endopeptidase activity; nucleotide binding; protein complex binding; phospholipid binding; calcium ion binding; catalytic activity; ATP binding; ligase activity

Biological Process: nitrogen compound metabolic process; glycogen catabolic process; arginine biosynthetic process; metabolic process; glutamine catabolic process; triacylglycerol catabolic process; response to lipopolysaccharide; homocysteine metabolic process; positive regulation of vasodilation; proteolysis; nitric oxide metabolic process; anion homeostasis; urea cycle

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