Names & Taxonomy

Uniprot ID:
P34897
Entry Name:
GLYM_HUMAN
Status:
reviewed
Protein Names:
Serine hydroxymethyltransferase, mitochondrial (SHMT) (EC 2.1.2.1) (Glycine hydroxymethyltransferase) (Serine methylase)
Gene Names:
SHMT2
Gene Names Primary:
SHMT2
Organism:
Homo sapiens (Human)

Structure

Length:
504
Sequence:
MLYFSLFWAARPLQRCGQLVRMAIRAQHSNAAQTQTGEANRGWTGQESLSDSDPEMWELLQREKDRQCRGLELIASENFCSRAALEALGSCLNNKYSEGYPGKRYYGGAEVVDEIELLCQRRALEAFDLDPAQWGVNVQPYSGSPANLAVYTALLQPHDRIMGLDLPDGGHLTHGYMSDVKRISATSIFFESMPYKLNPKTGLIDYNQLALTARLFRPRLIIAGTSAYARLIDYARMREVCDEVKAHLLADMAHISGLVAAKVIPSPFKHADIVTTTTHKTLRGARSGLIFYRKGVKAVDPKTGREIPYTFEDRINFAVFPSLQGGPHNHAIAAVAVALKQACTPMFREYSLQVLKNARAMADALLERGYSLVSGGTDNHLVLVDLRPKGLDGARAERVLELVSITANKNTCPGDRSAITPGGLRLGAPALTSRQFREDDFRRVVDFIDEGVNIGLEVKSKTAKLQDFKSFLLKDSETSQRLANLRQRVEQFARAFPMPGFDEH
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Mitochondrion

Function

Function:
Contributes to the de novo mitochondrial thymidylate biosynthesis pathway via its role in glycine and tetrahydrofolate metabolism. Thymidylate biosynthesis is required to prevent uracil accumulation in mtDNA (PubMed:21876188). Interconversion of serine and glycine (PubMed:25619277). Associates with mitochondrial DNA (PubMed:18063578). Plays a role in the deubiquitination of target proteins as component of the BRISC complex (PubMed:24075985). Required for IFNAR1 deubiquitination by the BRISC complex (PubMed:24075985).
Pathway:
One-carbon metabolism; tetrahydrofolate interconversion.
Catalytic Activity:
5,10-methylenetetrahydrofolate + glycine + H(2)O = tetrahydrofolate + L-serine.
Cofactor:
COFACTOR: Name=pyridoxal 5'-phosphate; Xref=ChEBI:CHEBI:597326;
Kinetics:
BIOPHYSICOCHEMICAL PROPERTIES: Kinetic parameters: KM=278 uM for L-serine
Cross Reference Drug Bank:
DB00145 DB00116
Gene Ontology Go:
BRISC complex
cytoplasm
extracellular exosome
microtubule cytoskeleton
mitochondrial inner membrane
mitochondrial intermembrane space
mitochondrial matrix
mitochondrial nucleoid
mitochondrion
nucleus
amino acid binding
chromatin binding
glycine hydroxymethyltransferase activity
L-allo-threonine aldolase activity
pyridoxal phosphate binding
folic acid metabolic process
glycine biosynthetic process from serine
glycine metabolic process
L-serine biosynthetic process
L-serine metabolic process
one-carbon metabolic process
positive regulation of cell proliferation
protein homotetramerization
protein K63-linked deubiquitination
protein tetramerization
response to type I interferon
small molecule metabolic process
tetrahydrofolate interconversion
tetrahydrofolate metabolic process
vitamin metabolic process
water-soluble vitamin metabolic process
Gene Ontology Biological Process:
folic acid metabolic process
glycine biosynthetic process from serine
glycine metabolic process
L-serine biosynthetic process
L-serine metabolic process
one-carbon metabolic process
positive regulation of cell proliferation
protein homotetramerization
protein K63-linked deubiquitination
protein tetramerization
response to type I interferon
small molecule metabolic process
tetrahydrofolate interconversion
tetrahydrofolate metabolic process
vitamin metabolic process
water-soluble vitamin metabolic process
Gene Ontology Molecular Function:
amino acid binding
chromatin binding
glycine hydroxymethyltransferase activity
L-allo-threonine aldolase activity
pyridoxal phosphate binding
Gene Ontology Cellular Component:
BRISC complex
cytoplasm
extracellular exosome
microtubule cytoskeleton
mitochondrial inner membrane
mitochondrial intermembrane space
mitochondrial matrix
mitochondrial nucleoid
mitochondrion
nucleus
Keywords:
3D-structure
Acetylation
Alternative splicing
Complete proteome
Cytoplasm
Membrane
Mitochondrion
Mitochondrion inner membrane
Mitochondrion nucleoid
Nucleus
One-carbon metabolism
Pyridoxal phosphate
Reference proteome
Transferase
Transit peptide
Interacts With:
Q15041; Q96DZ9

Publication

PubMed ID:
14702039 16541075 15489334 10828359 8999870 8505317 18063578 19608861 21269460 21876188 24075985 24275569 25902260 25944712 25619277