MTHFD1L - Monofunctional C1-tetrahydrofolate synthase, mitochondrial - human protein (Function)
 
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MTHFD1L »  Monofunctional C1-tetrahydrofolate synthase, mitochondrial   [ EC 6.3.4.3 ]
 
Protein also known as:  Formyltetrahydrofolate synthetase.
Gene name:  MTHFD1L
Family names: In the N-terminal section Tetrahydrofolate dehydrogenase/cyclohydrolase
In the C-terminal section Formate--tetrahydrofolate ligase
Entry whose protein(s) existence is based on evidence at protein level
extend overview
1 32 2
GENE REF ISO

Function

 show evidences
Overview 
May provide the missing metabolic reaction required to link the mitochondria and the cytoplasm in the mammalian model of one-carbon folate metabolism in embryonic an transformed cells complementing thus the enzymatic activities of MTHFD2 (By similarity).  
1
  • CuratedUniProtKB
GO molecular function 
ATP bindingdefinition[GO:0005524]  
1
  • IDABHF-UCL
Formate-tetrahydrofolate ligase activitydefinition[GO:0004329]  
1
  • IDABHF-UCL
Not  Methenyltetrahydrofolate cyclohydrolase activitydefinition[GO:0004477] silver  
  • IBARefGenome
Not  Methylenetetrahydrofolate dehydrogenase (NADP+) activitydefinition[GO:0004488] silver  
  • IBARefGenome
Protein homodimerization activitydefinition[GO:0042803]  
1
  • IDABHF-UCL
GO biological process 
Folic acid-containing compound biosynthetic processdefinition[GO:0009396] silver  
  • IEAInterPro 2 GO
Folic acid-containing compound metabolic processdefinition[GO:0006760]  
1
  • IDABHF-UCL
Formate metabolic processdefinition[GO:0015942]  
1
  • IDABHF-UCL
One-carbon metabolic processdefinition[GO:0006730] silver  
  • IBARefGenome
Tetrahydrofolate interconversiondefinition[GO:0035999]  
  • IEAUniPathway
Tetrahydrofolate metabolic processdefinition[GO:0046653]  
1
  • IDABHF-UCL
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reaction
EC 6.3.4.3: ATP + formate + tetrahydrofolate ADP + phosphate + 10-formyltetrahydrofolate.  
  • CuratedUniProtKB
 
More information is available from:
Pathways 
This protein is involved in the following pathway
According to KEGG, this protein belongs to the following pathways:
Metabolic pathways  hsa01100+25902  
One carbon pool by folate  hsa00670+25902  
Note 
May participate in the progression of colorectal cancer by conferring growth advantage. Could be a new molecular target for cancer therapy.  
  • CuratedUniProtKB
Caution 
This enzyme lacks methylenetetrahydrofolate dehydrogenase (EC 1.5.1.5) and ethenyltetrahydrofolate cyclohydrolase (EC 3.5.4.9) activities. An enzyme performing these two complementary activities has not been found in adult mitochondrial tissues; MTHFD2, which performs these two activities, was found in developing tissues only.  
  • CuratedUniProtKB
Was originally (PubMed12937168) thought to be a trifunctional enzyme but only a formyltetrahydrofolate synthetase activity was detected and not a dehydrogenase/cyclohydrogenase activity.  
1
  • CuratedUniProtKB
 

Biophysicochemical properties

Kinetic parameters
KM 500 uM for THF monoglutamate
KM 16 uM for THF triglutamate
KM 3.6 uM for THF pentaglutamate

Keywords

Biological process 
One-carbon metabolism  definition   [KW-0554]
Molecular function 
Ligase  definition   [KW-0436]
Technical term 
Reference proteome  definition   [KW-1185]
 

Further external links

GeneWiki: MTHFD1L
GenomeRNAi: 25902
PRO: PR:Q6UB35