InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: DMGDH

Summary

InnateDB Protein

IDBP-30781.6

Last Modified

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

Gene Symbol

DMGDH

Protein Name

dimethylglycine dehydrogenase

Synonyms

Species

Homo sapiens

Ensembl Protein

ENSP00000255189

InnateDB Gene

IDBG-30779 (DMGDH)

Protein Structure

UniProt Annotation

Function

Subcellular Localization

Mitochondrion.

Disease Associations

DMGDH deficiency (DMGDHD) [MIM:605850]: Disorder characterized by fish odor, muscle fatigue with increased serum creatine kinase. Biochemically it is characterized by an increase of N,N-dimethylglycine (DMG) in serum and urine. {ECO:0000269PubMed:11231903}. 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 0 experimentally validated interaction(s) in this database.

Gene Ontology

Molecular Function

Accession	GO Term
GO:0004047	aminomethyltransferase activity
GO:0009055	electron carrier activity
GO:0016491	oxidoreductase activity
GO:0044822	poly(A) RNA binding
GO:0047865	dimethylglycine dehydrogenase activity

Biological Process

GO:0006544	glycine metabolic process
GO:0006546	glycine catabolic process
GO:0006579	amino-acid betaine catabolic process
GO:0019695	choline metabolic process
GO:0032259	methylation
GO:0055114	oxidation-reduction process

Cellular Component

GO:0005739	mitochondrion
GO:0005759	mitochondrial matrix

Protein Structure and Domains

PDB ID

InterPro

IPR006076 FAD dependent oxidoreductase
IPR006222 Glycine cleavage T-protein, N-terminal
IPR007375 Sarcosine oxidase, gamma subunit
IPR013977 Glycine cleavage T-protein, C-terminal barrel domain
IPR029043 Glycine cleavage T-protein/YgfZ, C-terminal domain

PFAM

PF01266
PF01571
PF04268
PF08669

PRINTS

PIRSF

SMART

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

Q9UI17

PhosphoSite