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Information on EC 2.1.2.9 - methionyl-tRNA formyltransferase and Organism(s) Homo sapiens and UniProt Accession Q96DP5

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Homo sapiens
UNIPROT: Q96DP5 not found.
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The taxonomic range for the selected organisms is: Homo sapiens
The expected taxonomic range for this enzyme is: Bacteria, Eukaryota, Archaea
Synonyms
mtfmt, methionyl-trna formyltransferase, methionyl-trna transformylase, mitochondrial methionyl-trna formyltransferase, methionyl-trna-formyltransferase, fmt1p, formyl-methionyl transferase, more
SYNONYM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
mitochondrial methionyl-tRNA formyltransferase
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FMT
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formylmethionyl-transfer ribonucleic synthetase
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methionyl ribonucleic formyltransferase
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methionyl-transfer ribonucleic transformylase
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methionyl-transfer RNA transformylase
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methionyl-tRNA Met formyltransferase
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methionyl-tRNA transformylase
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methyltransferase, methionyl-transfer ribonucleate
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mitochondrial methionyl-tRNA formyltransferase
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MTF
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N10-formyltetrahydrofolic-methionyl-transfer ribonucleic transformylase
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
formyl group transfer
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SYSTEMATIC NAME
IUBMB Comments
10-formyltetrahydrofolate:L-methionyl-tRNA N-formyltransferase
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CAS REGISTRY NUMBER
COMMENTARY hide
9015-76-3
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SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
10-formyltetrahydrofolate + L-methionyl-tRNAfMet
tetrahydrofolate + N-formylmethionyl-tRNAfMet
show the reaction diagram
10-formyltetrahydrofolate + L-methionyl-tRNAfMet
tetrahydrofolate + N-formylmethionyl-tRNAfMet
show the reaction diagram
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-
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-
?
NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
10-formyltetrahydrofolate + L-methionyl-tRNAfMet
tetrahydrofolate + N-formylmethionyl-tRNAfMet
show the reaction diagram
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-
-
?
10-formyltetrahydrofolate + L-methionyl-tRNAfMet
tetrahydrofolate + N-formylmethionyl-tRNAfMet
show the reaction diagram
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?
COFACTOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
10-formyltetrahydrofolate
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METALS and IONS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
Mg2+
required
KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
additional information
additional information
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pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
7.6
assay at
TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
37
assay at
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
-
UniProt
Manually annotated by BRENDA team
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
malfunction
mutations of conserved enzyme residues affect enzyme activity and lead to poor formylation of mitochondrial methionyl-tRNA and thereby reduced mitochondrial translation efficiency, causing Leigh syndrome
physiological function
N-formylation of initiator methionyl-tRNA (Met-tRNAMet) by methionyl-tRNA formyltransferase is important for translation initiation in bacteria, mitochondria, and chloroplasts
malfunction
physiological function
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MTFMT is critical for efficient human mitochondrial translation
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION?
FMT_HUMAN
389
0
43832
Swiss-Prot
Mitochondrion (Reliability: 1)
PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
S125L
naturally occurring mutation, the mutant has a 653fold lower activity compared to the wild-type
S209L
naturally occurring mutation, the mutant has a 36fold lower activity compared to the wild-type
A59V
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the mutation causing substantially decreased enzyme activity is associated with selective vulnerability of the visual pathway and a mild neurological phenotype of Leigh syndrome. The missense mutation destabilizes the protein
R181S
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the mutation causes severe Leigh syndrome
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Tucker, E.J.; Hershman, S.G.; Koehrer, C.; Belcher-Timme, C.A.; Patel, J.; Goldberger, O.A.; Christodoulou, J.; Silberstein, J.M.; McKenzie, M.; Ryan, M.T.; Compton, A.G.; Jaffe, J.D.; Carr, S.A.; Calvo, S.E.; RajBhandary, U.L.; Thorburn, D.R.; Mootha, V.K.
Mutations in MTFMT underlie a human disorder of formylation causing impaired mitochondrial translation
Cell Metab.
14
428-434
2011
Homo sapiens
Manually annotated by BRENDA team
Sinha, A.; Koehrer, C.; Weber, M.H.; Masuda, I.; Mootha, V.K.; Hou, Y.M.; RajBhandary, U.L.
Biochemical characterization of pathogenic mutations in human mitochondrial methionyl-tRNA formyltransferase
J. Biol. Chem.
289
32729-32741
2014
Escherichia coli (P23882), Escherichia coli, Homo sapiens (Q96DP5), Homo sapiens
Manually annotated by BRENDA team
La Piana, R.; Weraarpachai, W.; Ospina, L.H.; Tetreault, M.; Majewski, J.; Majewski, J.; Bruce Pike, G.; Decarie, J.C.; Tampieri, D.; Brais, B.; Shoubridge, E.A.
Identification and functional characterization of a novel MTFMT mutation associated with selective vulnerability of the visual pathway and a mild neurological phenotype
Neurogenetics
18
97-103
2017
Homo sapiens
Manually annotated by BRENDA team