InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: GLDC

Summary

InnateDB Protein

IDBP-48716.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

GLDC

Protein Name

glycine dehydrogenase (decarboxylating)

Synonyms

GCE; GCSP; HYGN1;

Species

Homo sapiens

Ensembl Protein

ENSP00000370737

InnateDB Gene

IDBG-48712 (GLDC)

Protein Structure

UniProt Annotation

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).

Subcellular Localization

Mitochondrion.

Disease Associations

Non-ketotic hyperglycinemia (NKH) [MIM:605899]: Autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms. {ECO:0000269PubMed:11286506, ECO:0000269PubMed:11592811, ECO:0000269PubMed:1634607, ECO:0000269PubMed:1996985}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 7 experimentally validated interaction(s) in this database.

Experimentally validated
Total	7 [view]
Protein-Protein	7 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0004375	glycine dehydrogenase (decarboxylating) activity
GO:0009055	electron carrier activity
GO:0016829	lyase activity
GO:0030170	pyridoxal phosphate binding

Biological Process

GO:0006520	cellular amino acid metabolic process
GO:0006544	glycine metabolic process
GO:0006546	glycine catabolic process
GO:0055114	oxidation-reduction process

Cellular Component

GO:0005739

mitochondrion

Protein Structure and Domains

PDB ID

InterPro

IPR001597 Aromatic amino acid beta-eliminating lyase/threonine aldolase
IPR003437 Glycine cleavage system P protein, homodimeric
IPR015424 Pyridoxal phosphate-dependent transferase
IPR020580 Glycine cleavage system P-protein, N-terminal

PFAM

PF01212
PF02347

PRINTS

PIRSF

SMART

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

P23378

PhosphoSite