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Showing Protein Glycine N-methyltransferase (HMDBP00403)

IdentificationBiological propertiesGene propertiesProtein propertiesExternal linksReferencesXMLShow 6 metabolites
Identification
HMDB Protein ID HMDBP00403
Secondary Accession Numbers
  • 5640
  • HMDBP03600
Name Glycine N-methyltransferase
Synonyms Not Available
Gene Name GNMT
Protein Type Unknown
Biological Properties
General Function Involved in folic acid binding
Specific Function Catalyzes the methylation of glycine by using S-adenosylmethionine (AdoMet) to form N-methylglycine (sarcosine) with the concomitant production of S-adenosylhomocysteine (AdoHcy). Possible crucial role in the regulation of tissue concentration of AdoMet and of metabolism of methionine.
Pathways
  • 3-Phosphoglycerate dehydrogenase deficiency
  • Dihydropyrimidine Dehydrogenase Deficiency (DHPD)
  • Dimethylglycine Dehydrogenase Deficiency
  • Dimethylglycine Dehydrogenase Deficiency
  • Glycine, serine and threonine metabolism
  • Glycine, serine and threonine metabolism
  • Hyperglycinemia, non-ketotic
  • Non Ketotic Hyperglycinemia
  • Sarcosine Oncometabolite Pathway
  • Sarcosinemia
Reactions
S-Adenosylmethionine + Glycine → S-Adenosylhomocysteine + Sarcosine details
GO Classification
Biological Process
S-adenosylhomocysteine metabolic process
cellular protein modification process
glycogen metabolic process
S-adenosylmethionine metabolic process
protein homotetramerization
methionine metabolic process
one-carbon metabolic process
regulation of gluconeogenesis
Cellular Component
cytosol
cytoplasm
nucleus
Component
cell part
intracellular part
cytoplasm
Function
binding
catalytic activity
transferase activity
glycine n-methyltransferase activity
folic acid binding
carboxylic acid binding
amino acid binding
transferase activity, transferring one-carbon groups
methyltransferase activity
n-methyltransferase activity
Molecular Function
folic acid binding
glycine binding
glycine N-methyltransferase activity
Process
metabolic process
cellular metabolic process
sulfur amino acid metabolic process
methionine metabolic process
cellular amino acid and derivative metabolic process
cellular amino acid metabolic process
Cellular Location
  1. Cytoplasm
Gene Properties
Chromosome Location 6
Locus 6p12
SNPs GNMT
Gene Sequence 
>888 bp
ATGGTGGACAGCGTGTACCGGACCCGCTCCCTGGGGGTGGCGGCCGAAGGGCTCCCGGAC
CAGTACGCGGACGGGGAGGCGGCGCGCGTGTGGCAGCTGTATATCGGAGACACCCGCAGC
CGCACCGCCGAGTACAAGGCATGGCTGCTTGGGCTGCTGCGCCAGCACGGCTGCCAGCGG
GTGCTCGACGTAGCCTGTGGCACTGGGGTGGACTCCATTATGCTGGTGGAAGAGGGCTTC
AGTGTGACGAGTGTGGATGCCAGTGACAAGATGCTGAAGTATGCACTTAAGGAGCGCTGG
AACCGGCGGCACGAGCCCGCCTTCGACAAGTGGGTCATCGAAGAAGCCAACTGGATGACT
CTGGACAAAGATGTGCCCCAGTCAGCAGAGGGTGGCTTTGATGCTGTCATCTGCCTTGGA
AACAGTTTCGCTCACTTGCCAGACTGCAAAGGGGACCAGAGTGAGCACCGGCTGGCGCTG
AAAAACATTGCGAGCATGGTGCGGGCAGGGGGCCTACTGGTCATTGATCATCGCAACTAC
GACCACATCCTCAGTACAGGCTGTGCACCCCCAGGGAAGAACATCTACTATAAGAGTGAC
TTGACCAAGGACGTCACAACATCAGTGCTGATAGTGAACAACAAGGCCCACATGGTGACC
CTGGACTATACGGTGCAGGTGCCGGGGGCTGGCCAGGATGGCTCTCCTGGCTTGAGTAAG
TTCCGGCTCTCCTACTACCCACACTGTCTGGCATCCTTCACGGAGCTGCTCCAAGCAGCC
TTCGGAGGTAAGTGCCAGCACAGCGTCCTGGGCGACTTCAAGCCTTACAAGCCAGGCCAA
ACCTACATTCCCTGCTACTTCATCCACGTGCTCAAGAGGACAGACTGA
Protein Properties
Number of Residues 295
Molecular Weight 32742.0
Theoretical pI 7.023
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence 
>Glycine N-methyltransferase
MVDSVYRTRSLGVAAEGLPDQYADGEAARVWQLYIGDTRSRTAEYKAWLLGLLRQHGCQR
VLDVACGTGVDSIMLVEEGFSVTSVDASDKMLKYALKERWNRRHEPAFDKWVIEEANWMT
LDKDVPQSAEGGFDAVICLGNSFAHLPDCKGDQSEHRLALKNIASMVRAGGLLVIDHRNY
DHILSTGCAPPGKNIYYKSDLTKDVTTSVLIVNNKAHMVTLDYTVQVPGAGQDGSPGLSK
FRLSYYPHCLASFTELLQAAFGGKCQHSVLGDFKPYKPGQTYIPCYFIHVLKRTD
GenBank ID Protein 8671584
UniProtKB/Swiss-Prot ID Q14749
UniProtKB/Swiss-Prot Entry Name GNMT_HUMAN
PDB IDs
GenBank Gene ID AF101477
GeneCard ID GNMT
GenAtlas ID GNMT
HGNC ID HGNC:4415
References
General References
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  3. Chen YM, Shiu JY, Tzeng SJ, Shih LS, Chen YJ, Lui WY, Chen PH: Characterization of glycine-N-methyltransferase-gene expression in human hepatocellular carcinoma. Int J Cancer. 1998 Mar 2;75(5):787-93. [PubMed:9495250 ]
  4. Chen YM, Chen LY, Wong FH, Lee CM, Chang TJ, Yang-Feng TL: Genomic structure, expression, and chromosomal localization of the human glycine N-methyltransferase gene. Genomics. 2000 May 15;66(1):43-7. [PubMed:10843803 ]
  5. Ogawa H, Gomi T, Fujioka M: Mammalian glycine N-methyltransferases. Comparative kinetic and structural properties of the enzymes from human, rat, rabbit and pig livers. Comp Biochem Physiol B. 1993 Nov;106(3):601-11. [PubMed:8281755 ]
  6. Pakhomova S, Luka Z, Grohmann S, Wagner C, Newcomer ME: Glycine N-methyltransferases: a comparison of the crystal structures and kinetic properties of recombinant human, mouse and rat enzymes. Proteins. 2004 Nov 1;57(2):331-7. [PubMed:15340920 ]
  7. Luka Z, Pakhomova S, Luka Y, Newcomer ME, Wagner C: Destabilization of human glycine N-methyltransferase by H176N mutation. Protein Sci. 2007 Sep;16(9):1957-64. Epub 2007 Jul 27. [PubMed:17660255 ]
  8. Luka Z, Cerone R, Phillips JA 3rd, Mudd HS, Wagner C: Mutations in human glycine N-methyltransferase give insights into its role in methionine metabolism. Hum Genet. 2002 Jan;110(1):68-74. Epub 2001 Dec 7. [PubMed:11810299 ]
  9. Luka Z, Wagner C: Effect of naturally occurring mutations in human glycine N-methyltransferase on activity and conformation. Biochem Biophys Res Commun. 2003 Dec 26;312(4):1067-72. [PubMed:14651980 ]
  10. Augoustides-Savvopoulou P, Luka Z, Karyda S, Stabler SP, Allen RH, Patsiaoura K, Wagner C, Mudd SH: Glycine N -methyltransferase deficiency: a new patient with a novel mutation. J Inherit Metab Dis. 2003;26(8):745-59. [PubMed:14739680 ]