MTHFD2
"Nucleotide sequence of the human NAD-dependent methylene tetrahydrofolate dehydrogenase-cyclohydrolase". Nucleic Acids Res. 17 ... Bifunctional methylenetetrahydrofolate dehydrogenase/cyclohydrolase, mitochondrial is an enzyme that in humans is encoded by ... This gene encodes a nuclear-encoded mitochondrial bifunctional enzyme with methylenetetrahydrofolate dehydrogenase and ... Yang XM, MacKenzie RE (Nov 1993). "NAD-dependent methylenetetrahydrofolate dehydrogenase-methenyltetrahydrofolate ...
MTHFD1
"Nucleotide sequence of the human NAD-dependent methylene tetrahydrofolate dehydrogenase-cyclohydrolase". Nucleic Acids Research ... C-1-tetrahydrofolate synthase, cytoplasmic also known as C1-THF synthase is an enzyme that in humans is encoded by the MTHFD1 ( ... Allaire M, Li Y, MacKenzie RE, Cygler M (February 1998). "The 3-D structure of a folate-dependent dehydrogenase/cyclohydrolase ... Overview of all the structural information available in the PDB for UniProt: P11586 (C-1-tetrahydrofolate synthase, cytoplasmic ...
Formyltetrahydrofolate dehydrogenase
Other names in common use include 10-formyl tetrahydrofolate:NADP oxidoreductase, 10-formyl-H2PtGlu:NADP oxidoreductase, 10- ... formyl-H4folate dehydrogenase, N10-formyltetrahydrofolate dehydrogenase, and 10-formyltetrahydrofolate dehydrogenase. This ... In enzymology, a formyltetrahydrofolate dehydrogenase (EC 1.5.1.6) is an enzyme that catalyzes the chemical reaction 10- ... Kutzbach C, Stokstad EL (1968). "Partial purification of a 10-formyl-tetrahydrofolate: NADP oxidoreductase from mammalian liver ...
Formate-tetrahydrofolate ligase
... reverse reaction converting 10-formyltetrahydrofolate to tetrahydrofolate is performed by formyltetrahydrofolate dehydrogenase ... and tetrahydrofolate formylase. Human genes encoding formate-tetrahydrofolate ligases include: MTHFD1 - cytoplasmic MTHFD1L - ... I. The tetrahydrofolate formylase from pigeon liver. Purification and mechanism.]". Biochem. Z. 334: 108-32. PMID 13789141. ... The systematic name of this enzyme class is formate:tetrahydrofolate ligase (ADP-forming). Other names in common use include: ...
Histidine
Glutamate can then be deaminated by glutamate dehydrogenase or transaminated to form α-ketoglutarate. The histidine amino acid ... The formimino group is transferred to tetrahydrofolate, and the remaining five carbons form glutamate. Overall, these reactions ...
GCSH
... a tetrahydrofolate-requiring aminomethyltransferase enzyme), and L protein (a lipoamide dehydrogenase). The H protein shuttles ... a lipoamide dehydrogenase, catalyzes the oxidation of the dihydrolipoyl residue of H-protein and reduction of NAD. Human GCSH ... the release of ammonia and transfer of the one-carbon fragment from the intermediate lipoyl residue to tetrahydrofolate, while ...
Sarcosine dehydrogenase
Under the presence of tetrahydrofolate, sarcosine dehydrogenase binds to tetrahydrofolate and convert tetrahydrofolate to 5,10- ... Both sarcosine dehydrogenase and dimethylglycine dehydrogenase use FAD as a cofactor. Sarcosine dehydrogenase is linked by ... Sarcosinemia Sarcosine Dimethylglycine dehydrogenase Steenkamp DJ, Husain M (June 1982). "The effect of tetrahydrofolate on the ... FRISELL WR, MACKENZIE CG (1962). "Separation and purification of sarcosine dehydrogenase and dimethylglycine dehydrogenase". J ...
Dihydrofolate reductase
Miszta H, Dabrowski Z, Lanotte M (November 1988). "In vitro patterns of enzymic tetrahydrofolate dehydrogenase (EC 1.5.1.3) ... Found in all organisms, DHFR has a critical role in regulating the amount of tetrahydrofolate in the cell. Tetrahydrofolate and ... DHFR is responsible for the levels of tetrahydrofolate in a cell, and the inhibition of DHFR can limit the growth and ... In the end, dihydrofolate is reduced to tetrahydrofolate and NADPH is oxidized to NADP+. The high flexibility of Met20 and ...
10-Formyltetrahydrofolate
It can be converted back into tetrahydrofolate (THF) by formyltetrahydrofolate dehydrogenase or THF and formate by ... 10-Formyltetrahydrofolate (10-CHO-THF) is a form of tetrahydrofolate that acts as a donor of formyl groups in anabolism. In ... tetrahydrofolate ⇌ {\displaystyle \rightleftharpoons } ADP + phosphate + 10-formyltetrahydrofolate This reaction is catalyzed ... 10-Methylenetetrahydrofolate in Bifunctional Dehydrogenase-Cyclohydrolase Enzymes". Biochemistry. 37 (4): 1109-1115. doi: ...
MTHFD2L
... is a member of the tetrahydrofolate dehydrogenase/cyclohydrolase family. This enzyme is expressed in all adult tissues ... In the final step of this pathway, the NADP+-dependent CH2-THF dehydrogenase/CH+-THF cyclohydrolase activity of bifunctional ... NAD-dependent methylenetetrahydrofolate dehydrogenase 2-like protein (MTHFD2L), also known as bifunctional ... This gene encodes a 340-residue protein that is homologous to the mitochondrial bifunctional dehydrogenase/cyclohydrolase ( ...
ALDH1L1
10-formyltetrahydrofolate dehydrogenase is an enzyme that in humans is encoded by the ALDH1L1 gene. The protein encoded by this ... gene catalyzes the conversion of 10-formyltetrahydrofolate, NADP, and water to tetrahydrofolate, NADPH, and carbon dioxide. The ... "Entrez Gene: ALDH1L1 aldehyde dehydrogenase 1 family, member L1". Human ALDH1L1 genome location and ALDH1L1 gene details page ... Krupenko SA, Oleinik NV (2002). "10-formyltetrahydrofolate dehydrogenase, one of the major folate enzymes, is down-regulated in ...
ALDH1L2
Aldehyde dehydrogenase 1 family, member L2 also known as ALDH1L2 is an enzyme that in humans is encoded by the ALDH1L2 gene. ... which converts 10-formyltetrahydrofolate to tetrahydrofolate and carbon dioxide. GRCh38: Ensembl release 89: ENSG00000136010 - ... "ALDH1L2 is the mitochondrial homolog of 10-formyltetrahydrofolate dehydrogenase." J Biol Chem. 2010 Jul 23;285(30):23056-63. ... "Enzymatic properties of ALDH1L2, a mitochondrial 10-formyltetrahdyrofolate dehydrogenase." Chem Biol Interact. 2011 May 30;191( ...
5,10-Methenyltetrahydrofolate
... (5,10-CH=THF) is a form of tetrahydrofolate that is an intermediate in metabolism. 5,10-CH=THF is ... It is produced from 5,10-methylenetetrahydrofolate by either a NAD+ dependent methylenetetrahydrofolate dehydrogenase, or a ... NADP+ dependent dehydrogenase. It can also be produced as an intermediate in histidine catabolism, by formiminotransferase ...
MTHFD1L
... methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1-like). One-carbon substituted forms of tetrahydrofolate (THF) are ... "Entrez Gene: methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1-like". Christensen KE, Patel H, Kuzmanov U, Mejia NR, ... Monofunctional C1-tetrahydrofolate synthase, mitochondrial also known as formyltetrahydrofolate synthetase, is an enzyme that ... Sugiura T, Nagano Y, Inoue T, Hirotani K (2004). "A novel mitochondrial C1-tetrahydrofolate synthetase is upregulated in human ...
Glycine dehydrogenase (decarboxylating)
... a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). Glycine encephalopathy is due to defects in ... Glycine decarboxylase also known as glycine cleavage system P protein or glycine dehydrogenase is an enzyme that in humans is ... "Entrez Gene: GLDC glycine dehydrogenase (decarboxylating)". Kikuchi G (2008). "The glycine cleavage system: reaction mechanism ...
List of MeSH codes (D08)
... saccharopine dehydrogenases MeSH D08.811.682.662.825 - tetrahydrofolate dehydrogenase MeSH D08.811.682.664.249 - amine oxidase ... malate dehydrogenase MeSH D08.811.682.047.748 - malate dehydrogenase (nadp+) MeSH D08.811.682.047.892 - xanthine dehydrogenase ... acetoin dehydrogenase MeSH D08.811.682.047.070 - alcohol dehydrogenase MeSH D08.811.682.047.150 - carbohydrate dehydrogenases ... acyl-coa dehydrogenase MeSH D08.811.682.660.150.150 - acyl-coa dehydrogenase, long-chain MeSH D08.811.682.660.150.200 - acyl- ...
Lipoic acid
... the pyruvate dehydrogenase complex the α-ketoglutarate dehydrogenase or 2-oxoglutarate dehydrogenase complex the branched-chain ... and the T protein transfers the methylamine from lipoate to tetrahydrofolate (THF) yielding methylene-THF and ammonia. ... Lipoic acid is a cofactor for five enzymes or classes of enzymes: pyruvate dehydrogenase, a-ketoglutarate dehydrogenase, the ... oxoacid dehydrogenase (BCDH) complex the acetoin dehydrogenase complex. The most-studied of these is the pyruvate dehydrogenase ...
Riboflavin-responsive exercise intolerance
... dihydrolipoamide dehydrogenase. This dihydrolipoamide dehydrogenase dysfunction disrupted folate-mediated one-carbon metabolism ... may also assist with the conversion of tetrahydrofolate (THF) to 5,10-Methylenetetrahydrofolate (5,10-CH2-THF) a direct ... Multiple acyl-CoA dehydrogenase deficiency - similar in biochemical features; responsive to riboflavin in the majority of late- ...
Folate
Tetrahydrofolate's main function in metabolism is transporting single-carbon groups (i.e., a methyl group, methylene group, or ... A second enzyme, methylenetetrahydrofolate dehydrogenase (MTHFD2) oxidizes 5,10-methylenetetrahydrofolate to an iminium cation ... Alternative carbon sources include formate which by the catalytic action of formate-tetrahydrofolate ligase add a 1C unit to ... Folic acid is first reduced to dihydrofolate and then to tetrahydrofolate. Each step consumes one molecule of NADPH ( ...
Substrate-level phosphorylation
... tetrahydrofolate (reversibly), under both aerobic and anaerobic conditions. In the pay-off phase of glycolysis, a net of 2 ATP ... 3-bisphosphoglycerate via glyceraldehyde 3-phosphate dehydrogenase. 1,3-bisphosphoglycerate is then dephosphorylated via ... tetrahydrofolate. In working skeletal muscles and the brain, Phosphocreatine is stored as a readily available high-energy ... or monofunctional C1-tetrahydrofolate synthase. Mitochondrial phosphoenolpyruvate carboxykinase is thought to participate in ...
Mitochondrial folate transporter
The phenotype of the patients is reminiscent of multiple acyl-CoA dehydrogenase deficiency (MADD). According to a review ... The mitochondrial folate transporter (MTF) is a transport protein that facilitates the transfer of tetrahydrofolate across the ...
Biological carbon fixation
The Carbon Monoxide Dehydrogenase/Acetyl-CoA Synthase is the oxygen-sensitive enzyme that permits the reduction of CO2 to CO ... The intermediates are formate for bacteria and formyl-methanofuran for archaea, and also the carriers, tetrahydrofolate and ... 6-phosphogluconate dehydrogenase catalyzes the reductive carboxylation of ribulose 5-phosphate to 6-phosphogluconate in E. coli ... Schauder R, Preuß A, Jetten M, Fuchs G (1989). "Oxidative and reductive acetyl CoA/carbon monoxide dehydrogenase pathway in ...
Glycine
... tetrahydrofolate → glycine + N5,N10-methylene tetrahydrofolate + H2O In E. coli, glycine is sensitive to antibiotics that ... Glyoxylate is then oxidized by hepatic lactate dehydrogenase to oxalate in an NAD+-dependent reaction. The half-life of glycine ... N10-methylene tetrahydrofolate + NADH + H+ ⇌ Glycine + tetrahydrofolate + NAD+ In addition to being synthesized from serine, ... In this context, the enzyme system involved is usually called the glycine cleavage system: Glycine + tetrahydrofolate + NAD+ ⇌ ...
Serine
At present three disorders have been reported: 3-phosphoglycerate dehydrogenase deficiency 3-phosphoserine phosphatase ... 8-tetrahydrofolate to 5,10-methylenetetrahydrofolate (mTHF) (hydrolysis). SHMT is a pyridoxal phosphate (PLP) dependent enzyme ... in patients with 3-phosphoglycerate dehydrogenase deficiency to intractable seizures. These symptoms respond to a variable ... to 3-phosphohydroxypyruvate and NADH by phosphoglycerate dehydrogenase (EC 1.1.1.95). Reductive amination (transamination) of ...
Biosynthesis
Aspartate-semialdehyde dehydrogenase catalyzes the reduction reaction by dephosphorylation of aspartyl-β-phosphate to yield ... 10-methylene-tetrahydrofolate. Cysteine biosynthesis is a two-step reaction that involves the incorporation of inorganic sulfur ... Aspartate semialdehyde dehydrogenase catalyzes the NADPH-dependent reduction of aspartyl phosphate to yield aspartate ... The other pathway for incorporating nitrogen onto the α-carbon of amino acids involves the enzyme glutamate dehydrogenase (GDH ...
Trimethylglycine
"BRENDA - Information on EC 1.2.1.8 - betaine-aldehyde dehydrogenase". Brenda-enzymes.org. Archived from the original on 29 June ... 10-methylene-tetrahydrofolate reductase (MTHFR), or cobalamin cofactor metabolism (cbl). The most common side effect is ... Chern, M. K.; Pietruszko, R. (1999). "Evidence for mitochondrial localization of betaine aldehyde dehydrogenase in rat liver: ... Further degradation of betaine, via the enzyme dimethylglycine dehydrogenase produces folate, thus contributing back to ...
Choline
This is oxidized by mitochondrial or cytosolic betaine-aldehyde dehydrogenases to trimethylglycine. Trimethylglycine is a ... tetrahydrofolate receives one of the methyl groups). Methylglycine forms, is excreted into urine, or is demethylated to glycine ...
Glycine cleavage system
dihydrolipoamide dehydrogenase lipoic acid glycine encephalopathy Kikuchi G (June 1973). "The glycine cleavage system: ... The H-protein forms a complex with the T-protein that uses tetrahydrofolate and yields ammonia and 5,10- ...
UDP-4-amino-4-deoxy-L-arabinose formyltransferase
8-tetrahydrofolate + UDP-4-deoxy-4-formamido-beta-L-arabinopyranose The activity is part of a bifunctional enzyme that also ... conformational change implies ordered dehydrogenase mechanism in key enzyme for polymyxin resistance". Structure. 13 (6): 929- ...
Calvin cycle
Via the glycine cleavage system and tetrahydrofolate, two glycines are converted into serine plus CO 2. Serine can be converted ... Insights from Transgenic Plants with Reduced Amounts of Cytochrome b/f Complex or Glyceraldehyde 3-Phosphate Dehydrogenase". ... The thioredoxin/ferredoxin system activates the enzymes glyceraldehyde-3-P dehydrogenase, glyceraldehyde-3-P phosphatase, ... The enzyme glyceraldehyde 3-phosphate dehydrogenase catalyses the reduction of 1,3BPGA by NADPH (which is another product of ...