Names & Taxonomy
- Uniprot ID:
- P23378
- Entry Name:
- GCSP_HUMAN
- Status:
- reviewed
- Protein Names:
- Glycine dehydrogenase (decarboxylating), mitochondrial (EC 1.4.4.2) (Glycine cleavage system P protein) (Glycine decarboxylase) (Glycine dehydrogenase (aminomethyl-transferring))
- Gene Names:
- GLDC GCSP
- Gene Names Primary:
- GLDC
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 1020
- Sequence:
- MQSCARAWGLRLGRGVGGGRRLAGGSGPCWAPRSRDSSSGGGDSAAAGASRLLERLLPRHDDFARRHIGPGDKDQREMLQTLGLASIDELIEKTVPANIRLKRPLKMEDPVCENEILATLHAISSKNQIWRSYIGMGYYNCSVPQTILRNLLENSGWITQYTPYQPEVSQGRLESLLNYQTMVCDITGLDMANASLLDEGTAAAEALQLCYRHNKRRKFLVDPRCHPQTIAVVQTRAKYTGVLTELKLPCEMDFSGKDVSGVLFQYPDTEGKVEDFTELVERAHQSGSLACCATDLLALCILRPPGEFGVDIALGSSQRFGVPLGYGGPHAAFFAVRESLVRMMPGRMVGVTRDATGKEVYRLALQTREQHIRRDKATSNICTAQALLANMAAMFAIYHGSHGLEHIARRVHNATLILSEGLKRAGHQLQHDLFFDTLKIQCGCSVKEVLGRAAQRQINFRLFEDGTLGISLDETVNEKDLDDLLWIFGCESSAELVAESMGEECRGIPGSVFKRTSPFLTHQVFNSYHSETNIVRYMKKLENKDISLVHSMIPLGSCTMKLNSSSELAPITWKEFANIHPFVPLDQAQGYQQLFRELEKDLCELTGYDQVCFQPNSGAQGEYAGLATIRAYLNQKGEGHRTVCLIPKSAHGTNPASAHMAGMKIQPVEVDKYGNIDAVHLKAMVDKHKENLAAIMITYPSTNGVFEENISDVCDLIHQHGGQVYLDGANMNAQVGICRPGDFGSDVSHLNLHKTFCIPHGGGGPGMGPIGVKKHLAPFLPNHPVISLKRNEDACPVGTVSAAPWGSSSILPISWAYIKMMGGKGLKQATETAILNANYMAKRLETHYRILFRGARGYVGHEFILDTRPFKKSANIEAVDVAKRLQDYGFHAPTMSWPVAGTLMVEPTESEDKAELDRFCDAMISIRQEIADIEEGRIDPRVNPLKMSPHSLTCVTSSHWDRPYSREVAAFPLPFVKPENKFWPTIARIDDIYGDQHLVCTCPPMEVYESPFSEQKRASS
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion.
Function
- Function:
- The glycine cleavage system catalyzes the degradation of glycine. The P protein (GLDC) binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor; CO(2) is released and the remaining methylamine moiety is then transferred to the lipoamide cofactor of the H protein (GCSH).
- Catalytic Activity:
- Glycine + -N(6)-lipoyl-L-lysine = -S-aminomethyl-N(6)-dihydrolipoyl-L-lysine + CO(2).
- Cofactor:
- COFACTOR: Name=pyridoxal 5'-phosphate; Xref=ChEBI:CHEBI:597326;
- Enzyme Regulation:
- ENZYME REGULATION: Stimulated by lipoic acid. Inhibited in presence of methylamine (By similarity).
- Cross Reference Drug Bank:
- DB00145
- Gene Ontology Go:
- glycine cleavage complex
mitochondrial matrix
mitochondrion
nucleus
plasma membrane
electron carrier activity
glycine binding
glycine dehydrogenase (decarboxylating) activity
lyase activity
cellular nitrogen compound metabolic process
glycine catabolic process
glycine decarboxylation via glycine cleavage system
glyoxylate metabolic process
small molecule metabolic process - Gene Ontology Biological Process:
- cellular nitrogen compound metabolic process
glycine catabolic process
glycine decarboxylation via glycine cleavage system
glyoxylate metabolic process
small molecule metabolic process - Gene Ontology Molecular Function:
- electron carrier activity
glycine binding
glycine dehydrogenase (decarboxylating) activity
lyase activity - Gene Ontology Cellular Component:
- glycine cleavage complex
mitochondrial matrix
mitochondrion
nucleus
plasma membrane - Keywords:
- Acetylation
Complete proteome
Disease mutation
Mitochondrion
Oxidoreductase
Pyridoxal phosphate
Reference proteome
Transit peptide