Names & Taxonomy
- Uniprot ID:
- P11498
- Entry Name:
- PYC_HUMAN
- Status:
- reviewed
- Protein Names:
- Pyruvate carboxylase, mitochondrial (EC 6.4.1.1) (Pyruvic carboxylase) (PCB)
- Gene Names:
- PC
- Gene Names Primary:
- PC
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 1178
- Sequence:
- MLKFRTVHGGLRLLGIRRTSTAPAASPNVRRLEYKPIKKVMVANRGEIAIRVFRACTELGIRTVAIYSEQDTGQMHRQKADEAYLIGRGLAPVQAYLHIPDIIKVAKENNVDAVHPGYGFLSERADFAQACQDAGVRFIGPSPEVVRKMGDKVEARAIAIAAGVPVVPGTDAPITSLHEAHEFSNTYGFPIIFKAAYGGGGRGMRVVHSYEELEENYTRAYSEALAAFGNGALFVEKFIEKPRHIEVQILGDQYGNILHLYERDCSIQRRHQKVVEIAPAAHLDPQLRTRLTSDSVKLAKQVGYENAGTVEFLVDRHGKHYFIEVNSRLQVEHTVTEEITDVDLVHAQIHVAEGRSLPDLGLRQENIRINGCAIQCRVTTEDPARSFQPDTGRIEVFRSGEGMGIRLDNASAFQGAVISPHYDSLLVKVIAHGKDHPTAATKMSRALAEFRVRGVKTNIAFLQNVLNNQQFLAGTVDTQFIDENPELFQLRPAQNRAQKLLHYLGHVMVNGPTTPIPVKASPSPTDPVVPAVPIGPPPAGFRDILLREGPEGFARAVRNHPGLLLMDTTFRDAHQSLLATRVRTHDLKKIAPYVAHNFSKLFSMENWGGATFDVAMRFLYECPWRRLQELRELIPNIPFQMLLRGANAVGYTNYPDNVVFKFCEVAKENGMDVFRVFDSLNYLPNMLLGMEAAGSAGGVVEAAISYTGDVADPSRTKYSLQYYMGLAEELVRAGTHILCIKDMAGLLKPTACTMLVSSLRDRFPDLPLHIHTHDTSGAGVAAMLACAQAGADVVDVAADSMSGMTSQPSMGALVACTRGTPLDTEVPMERVFDYSEYWEGARGLYAAFDCTATMKSGNSDVYENEIPGGQYTNLHFQAHSMGLGSKFKEVKKAYVEANQMLGDLIKVTPSSKIVGDLAQFMVQNGLSRAEAEAQAEELSFPRSVVEFLQGYIGVPHGGFPEPFRSKVLKDLPRVEGRPGASLPPLDLQALEKELVDRHGEEVTPEDVLSAAMYPDVFAHFKDFTATFGPLDSLNTRLFLQGPKIAEEFEVELERGKTLHIKALAVSDLNRAGQRQVFFELNGQLRSILVKDTQAMKEMHFHPKALKDVKGQIGAPMPGKVIDIKVVAGAKVAKGQPLCVLSAMKMETVVTSPMEGTVRKVHVTKDMTLEGDDLILEIE
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion matrix.
Function
- Function:
- Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate.
- Pathway:
- Carbohydrate biosynthesis; gluconeogenesis.
- Catalytic Activity:
- ATP + pyruvate + HCO(3)(-) = ADP + phosphate + oxaloacetate.
- Cofactor:
- COFACTOR: Name=biotin; Xref=ChEBI:CHEBI:57586; ; COFACTOR: Name=Mn(2+); Xref=ChEBI:CHEBI:29035; ; Note=Binds 1 Mn(2+) ion per subunit.;
- Active Site:
- ACT_SITE 328 328
- Cross Reference Drug Bank:
- DB00121 DB00119
- Gene Ontology Go:
- cytoplasm
cytosol
mitochondrial matrix
mitochondrion
ATP binding
biotin binding
biotin carboxylase activity
DNA binding
metal ion binding
pyruvate carboxylase activity
biotin metabolic process
carbohydrate metabolic process
gluconeogenesis
glucose metabolic process
lipid metabolic process
negative regulation of gene expression
oxaloacetate metabolic process
positive regulation by host of viral process
positive regulation by host of viral release from host cell
pyruvate metabolic process
small molecule metabolic process
viral RNA genome packaging
vitamin metabolic process
water-soluble vitamin metabolic process - Gene Ontology Biological Process:
- biotin metabolic process
carbohydrate metabolic process
gluconeogenesis
glucose metabolic process
lipid metabolic process
negative regulation of gene expression
oxaloacetate metabolic process
positive regulation by host of viral process
positive regulation by host of viral release from host cell
pyruvate metabolic process
small molecule metabolic process
viral RNA genome packaging
vitamin metabolic process
water-soluble vitamin metabolic process - Gene Ontology Molecular Function:
- ATP binding
biotin binding
biotin carboxylase activity
DNA binding
metal ion binding
pyruvate carboxylase activity - Gene Ontology Cellular Component:
- cytoplasm
cytosol
mitochondrial matrix
mitochondrion - Keywords:
- 3D-structure
ATP-binding
Acetylation
Alternative splicing
Biotin
Complete proteome
Disease mutation
Gluconeogenesis
Ligase
Lipid biosynthesis
Lipid metabolism
Manganese
Metal-binding
Mitochondrion
Multifunctional enzyme
Nucleotide-binding
Phosphoprotein
Polymorphism
Pyruvate
Reference proteome
Transit peptide - Interacts With:
- Q03463