| Identification |
|---|
| HMDB Protein ID
| HMDBP12557 |
| Secondary Accession Numbers
| None |
| Name
| Carbamoyl-phosphate synthase [ammonia], mitochondrial |
| Synonyms
|
- Carbamoyl-phosphate synthetase I
- CPSase I
|
| Gene Name
| CPS1 |
| Protein Type
| Unknown |
| Biological Properties |
|---|
| General Function
| Not Available |
| Specific Function
| Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell. |
| Pathways
|
- Alanine, aspartate and glutamate metabolism
- Arginine biosynthesis
- Biosynthesis of amino acids
- Carbon metabolism
- Nitrogen metabolism
|
| Reactions
| Not Available |
| GO Classification
|
| Biological Process |
| vasodilation |
| response to amine stimulus |
| urea cycle |
| glutamine metabolic process |
| cellular response to ammonium ion |
| cellular response to cAMP |
| cellular response to oleic acid |
| response to growth hormone stimulus |
| nitrogen compound metabolic process |
| response to steroid hormone stimulus |
| response to glucagon stimulus |
| response to zinc ion |
| cellular response to fibroblast growth factor stimulus |
| hepatocyte differentiation |
| response to food |
| anion homeostasis |
| carbamoyl phosphate biosynthetic process |
| homocysteine metabolic process |
| nitric oxide metabolic process |
| response to dexamethasone stimulus |
| 'de novo' pyrimidine nucleobase biosynthetic process |
| response to amino acid stimulus |
| liver development |
| response to drug |
| response to oleic acid |
| response to starvation |
| response to glucocorticoid stimulus |
| triglyceride catabolic process |
| cellular response to glucagon stimulus |
| response to lipopolysaccharide |
| midgut development |
| response to cAMP |
| response to toxin |
| Cellular Component |
| mitochondrial nucleoid |
| protein-containing complex |
| mitochondrial inner membrane |
| nucleolus |
| cytoplasm |
| mitochondrion |
| Molecular Function |
| glutamate binding |
| protein-containing complex binding |
| carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity |
| ATP binding |
| phospholipid binding |
| carbamoyl-phosphate synthase (ammonia) activity |
| calcium ion binding |
| modified amino acid binding |
| endopeptidase activity |
|
| Cellular Location
|
Not Available
|
| Gene Properties |
|---|
| Chromosome Location
| Not Available |
| Locus
| Not Available |
| SNPs
| Not Available |
| Gene Sequence
|
Not Available
|
| Protein Properties |
|---|
| Number of Residues
| 1500 |
| Molecular Weight
| 164578.575 |
| Theoretical pI
| 6.769 |
| Pfam Domain Function
|
|
| Signals
|
Not Available
|
|
Transmembrane Regions
|
Not Available
|
| Protein Sequence
|
Not Available
|
| External Links |
|---|
| GenBank ID Protein
| Not Available |
| UniProtKB/Swiss-Prot ID
| P07756 |
| UniProtKB/Swiss-Prot Entry Name
| CPSM_RAT |
| PDB IDs
|
Not Available |
| GenBank Gene ID
| Not Available |
| GeneCard ID
| Not Available |
| GenAtlas ID
| Not Available |
| HGNC ID
| Not Available |
| References |
|---|
| General References
| - Lundby A, Secher A, Lage K, Nordsborg NB, Dmytriyev A, Lundby C, Olsen JV: Quantitative maps of protein phosphorylation sites across 14 different rat organs and tissues. Nat Commun. 2012 Jun 6;3:876. doi: 10.1038/ncomms1871. [PubMed:22673903 ]
- Nyunoya H, Broglie KE, Widgren EE, Lusty CJ: Characterization and derivation of the gene coding for mitochondrial carbamyl phosphate synthetase I of rat. J Biol Chem. 1985 Aug 5;260(16):9346-56. [PubMed:2991241 ]
- Lagace M, Howell BW, Burak R, Lusty CJ, Shore GC: Rat carbamyl-phosphate synthetase I gene. Promoter sequence and tissue-specific transcriptional regulation in vitro. J Biol Chem. 1987 Aug 5;262(22):10415-8. [PubMed:3038878 ]
- Pekkala S, Martinez AI, Barcelona B, Gallego J, Bendala E, Yefimenko I, Rubio V, Cervera J: Structural insight on the control of urea synthesis: identification of the binding site for N-acetyl-L-glutamate, the essential allosteric activator of mitochondrial carbamoyl phosphate synthetase. Biochem J. 2009 Nov 11;424(2):211-20. doi: 10.1042/BJ20090888. [PubMed:19754428 ]
- Diez-Fernandez C, Martinez AI, Pekkala S, Barcelona B, Perez-Arellano I, Guadalajara AM, Summar M, Cervera J, Rubio V: Molecular characterization of carbamoyl-phosphate synthetase (CPS1) deficiency using human recombinant CPS1 as a key tool. Hum Mutat. 2013 Aug;34(8):1149-59. doi: 10.1002/humu.22349. Epub 2013 May 28. [PubMed:23649895 ]
- Cao W, Cao J, Huang J, Yao J, Yan G, Xu H, Yang P: Discovery and confirmation of O-GlcNAcylated proteins in rat liver mitochondria by combination of mass spectrometry and immunological methods. PLoS One. 2013 Oct 2;8(10):e76399. doi: 10.1371/journal.pone.0076399. eCollection 2013. [PubMed:24098488 ]
|
