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Protein

Monofunctional C1-tetrahydrofolate synthase, mitochondrial

Gene

Mthfd1l

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

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 MTHFD2.1 Publication

Catalytic activityi

ATP + formate + tetrahydrofolate = ADP + phosphate + 10-formyltetrahydrofolate.

Pathwayi: tetrahydrofolate interconversion

This protein is involved in the pathway tetrahydrofolate interconversion, which is part of One-carbon metabolism.
View all proteins of this organism that are known to be involved in the pathway tetrahydrofolate interconversion and in One-carbon metabolism.

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi422 – 429ATPBy similarity8

GO - Molecular functioni

GO - Biological processi

  • 10-formyltetrahydrofolate biosynthetic process Source: MGI
  • embryonic neurocranium morphogenesis Source: BHF-UCL
  • embryonic viscerocranium morphogenesis Source: BHF-UCL
  • folic acid-containing compound metabolic process Source: MGI
  • formate metabolic process Source: MGI
  • neural tube closure Source: BHF-UCL
  • one-carbon metabolic process Source: GO_Central
  • oxidation-reduction process Source: InterPro
  • tetrahydrofolate interconversion Source: UniProtKB-UniPathway
  • tetrahydrofolate metabolic process Source: MGI
Complete GO annotation...

Keywords - Molecular functioni

Ligase

Keywords - Biological processi

One-carbon metabolism

Keywords - Ligandi

ATP-binding, Nucleotide-binding

Enzyme and pathway databases

BRENDAi6.3.4.3. 3474.
ReactomeiR-MMU-196757. Metabolism of folate and pterines.
UniPathwayiUPA00193.

Names & Taxonomyi

Protein namesi
Recommended name:
Monofunctional C1-tetrahydrofolate synthase, mitochondrial (EC:6.3.4.3)
Alternative name(s):
Formyltetrahydrofolate synthetase
Gene namesi
Name:Mthfd1l
Synonyms:Fthfsdc1
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 10

Organism-specific databases

MGIiMGI:1924836. Mthfd1l.

Subcellular locationi

GO - Cellular componenti

  • membrane Source: MGI
  • mitochondrion Source: BHF-UCL
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 31MitochondrionSequence analysisAdd BLAST31
ChainiPRO_000034317832 – 977Monofunctional C1-tetrahydrofolate synthase, mitochondrialAdd BLAST946

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei188N6-acetyllysine; alternateBy similarity1
Modified residuei188N6-succinyllysine; alternateCombined sources1
Modified residuei356PhosphoserineBy similarity1
Modified residuei595N6-succinyllysineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiQ3V3R1.
MaxQBiQ3V3R1.
PaxDbiQ3V3R1.
PeptideAtlasiQ3V3R1.
PRIDEiQ3V3R1.

2D gel databases

REPRODUCTION-2DPAGEQ3V3R1.

PTM databases

iPTMnetiQ3V3R1.
PhosphoSitePlusiQ3V3R1.
SwissPalmiQ3V3R1.

Expressioni

Gene expression databases

BgeeiENSMUSG00000040675.
GenevisibleiQ3V3R1. MM.

Interactioni

Subunit structurei

Homodimer.By similarity

GO - Molecular functioni

Protein-protein interaction databases

BioGridi234813. 2 interactors.
IntActiQ3V3R1. 9 interactors.
MINTiMINT-4114903.
STRINGi10090.ENSMUSP00000036178.

Structurei

Secondary structure

1977
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi514 – 521Combined sources8
Beta strandi525 – 528Combined sources4
Helixi533 – 542Combined sources10
Turni549 – 551Combined sources3
Helixi554 – 562Combined sources9

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2EO2NMR-A510-573[»]
ProteinModelPortaliQ3V3R1.
SMRiQ3V3R1.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ3V3R1.

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni32 – 347Methylenetetrahydrofolate dehydrogenase and cyclohydrolaseAdd BLAST316
Regioni348 – 977Formyltetrahydrofolate synthetaseAdd BLAST630

Domaini

This monofunctional enzyme consists of two major domains: an N-terminal inactive methylene-THF dehydrogenase and cyclohydrolase domain and an active larger formyl-THF synthetase C-terminal domain.

Sequence similaritiesi

In the N-terminal section; belongs to the tetrahydrofolate dehydrogenase/cyclohydrolase family.Curated
In the C-terminal section; belongs to the formate--tetrahydrofolate ligase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG4230. Eukaryota.
COG0190. LUCA.
COG2759. LUCA.
GeneTreeiENSGT00860000133777.
HOGENOMiHOG000040280.
HOVERGENiHBG004916.
InParanoidiQ3V3R1.
KOiK13402.
OMAiCSHDFLS.
OrthoDBiEOG091G01BU.
PhylomeDBiQ3V3R1.
TreeFamiTF300623.

Family and domain databases

CDDicd00477. FTHFS. 1 hit.
Gene3Di3.40.50.300. 2 hits.
3.40.50.720. 2 hits.
HAMAPiMF_01543. FTHFS. 1 hit.
InterProiIPR000559. Formate_THF_ligase.
IPR020628. Formate_THF_ligase_CS.
IPR016040. NAD(P)-bd_dom.
IPR027417. P-loop_NTPase.
IPR000672. THF_DH/CycHdrlase.
IPR020630. THF_DH/CycHdrlase_cat_dom.
IPR020631. THF_DH/CycHdrlase_NAD-bd_dom.
[Graphical view]
PfamiPF01268. FTHFS. 1 hit.
PF00763. THF_DHG_CYH. 1 hit.
PF02882. THF_DHG_CYH_C. 1 hit.
[Graphical view]
PRINTSiPR00085. THFDHDRGNASE.
SUPFAMiSSF51735. SSF51735. 1 hit.
SSF52540. SSF52540. 2 hits.
PROSITEiPS00721. FTHFS_1. 1 hit.
PS00722. FTHFS_2. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

Q3V3R1-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MSVRLPLLLR QLGRQQLPSG PACRLRELCR SGSRSSSSGG GDPEGLRGRR
60 70 80 90 100
LQDGQTFSSH GPGNPEAPGM DSIVRDVIHN SKEVLSLLQE KNPAFKPVLV
110 120 130 140 150
VIQAGDDNLM KDMNQNLAKE AGLDITHICL PPDSGEDEII DEILKINEDP
160 170 180 190 200
RVHGLTLQIS EDSLSNKVLN ALKPEKDVDG VTDINLGKLV RGDAPECFVS
210 220 230 240 250
PLAKAAVELV EKSGITLDGK KVLVVGAHGP LEAALQWLFQ RKGSMTMSCP
260 270 280 290 300
WATPQLPDKL READIVVLGS PKPEEIPAVW IPSGTTILNC FHDFLSGKLS
310 320 330 340 350
GGSPGVPVDK LIAEESVSLL AAALRIQNMV SSGRRWLREQ QHRRWRLHCL
360 370 380 390 400
KLQPLSPVPS DIEISRGQTP KAVDVLAKEI GLLADEIEIY GKSKAKVQLS
410 420 430 440 450
LLERLKDQTD GKYVLVAGIT PTPLGEGKST VTIGLVQALT AHLKVNSFAC
460 470 480 490 500
LRQPSQGPTF GVKGGAAGGG YAQVIPMEEF NLHLTGDIHA ITAANNLLAA
510 520 530 540 550
AIDTRILHES TQTDKALYNR LVPLVNGVRE FSEIQLSRLK KLGIHKTDPS
560 570 580 590 600
TLTEEEVRKF ARLNIDPATI TWQRVLDTND RFLRKITIGQ GSTEKGYSRQ
610 620 630 640 650
AQFDIAVASE IMAVLALTDS LTDMKERLGR MVVASDKDGQ PVTAEDLGVT
660 670 680 690 700
GALTVLMKDA IKPNLMQTLE GTPVFVHAGP FANIAHGNSS VLADKIALKL
710 720 730 740 750
VGEEGFVVTE AGFGADIGME KFFNIKCRAS GLVPNVVVLV ATVRALKMHG
760 770 780 790 800
GGPSVTAGVP LKKEYTEENI QLVADGCCNL QKQIQIAQLF GVPVVVALNV
810 820 830 840 850
FKTDTRAEID LVCELAKRAG AFDAVPCYHW SAGGKGSVDL ARAVREAANK
860 870 880 890 900
RSRFQFLYDV QLPIVEKIRV IAQTVYGAKD IELSPEAQSK IDRYTQQGFG
910 920 930 940 950
NLPICMAKTH LSLSHEPDKK GVPRDFTLPI SDVRASIGAG FIYPLVGTMS
960 970
TMPGLPTRPC FYDIDLDTET EQVKGLF
Length:977
Mass (Da):105,729
Last modified:July 1, 2008 - v2
Checksum:i7275EF6F9A4292D6
GO

Sequence cautioni

The sequence AAH30437 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti137D → N in BAE35568 (PubMed:16141072).Curated1
Sequence conflicti279V → A in BAE35568 (PubMed:16141072).Curated1
Sequence conflicti279V → A in AAH30437 (PubMed:15489334).Curated1
Sequence conflicti279V → A in AAH49936 (PubMed:15489334).Curated1
Sequence conflicti353Q → H in BAE35568 (PubMed:16141072).Curated1
Sequence conflicti369T → N in BAE20500 (PubMed:16141072).Curated1
Sequence conflicti419I → V in BAC30053 (PubMed:16141072).Curated1
Sequence conflicti422T → S in BAE35568 (PubMed:16141072).Curated1
Sequence conflicti632V → A in BAE35568 (PubMed:16141072).Curated1
Sequence conflicti963D → G in BAE20438 (PubMed:16141072).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK038579 mRNA. Translation: BAC30053.1.
AK028211 mRNA. Translation: BAE20438.1.
AK036284 mRNA. Translation: BAE20500.1.
AK160025 mRNA. Translation: BAE35568.1.
BC030437 mRNA. Translation: AAH30437.1. Different initiation.
BC049936 mRNA. Translation: AAH49936.1.
CCDSiCCDS56672.1.
RefSeqiNP_001164256.1. NM_001170785.1.
NP_001164257.1. NM_001170786.1.
NP_758512.3. NM_172308.4.
UniGeneiMm.184752.

Genome annotation databases

EnsembliENSMUST00000043735; ENSMUSP00000036178; ENSMUSG00000040675.
ENSMUST00000117291; ENSMUSP00000112870; ENSMUSG00000040675.
ENSMUST00000120585; ENSMUSP00000112897; ENSMUSG00000040675.
GeneIDi270685.
KEGGimmu:270685.
UCSCiuc007ehj.2. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK038579 mRNA. Translation: BAC30053.1.
AK028211 mRNA. Translation: BAE20438.1.
AK036284 mRNA. Translation: BAE20500.1.
AK160025 mRNA. Translation: BAE35568.1.
BC030437 mRNA. Translation: AAH30437.1. Different initiation.
BC049936 mRNA. Translation: AAH49936.1.
CCDSiCCDS56672.1.
RefSeqiNP_001164256.1. NM_001170785.1.
NP_001164257.1. NM_001170786.1.
NP_758512.3. NM_172308.4.
UniGeneiMm.184752.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2EO2NMR-A510-573[»]
ProteinModelPortaliQ3V3R1.
SMRiQ3V3R1.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi234813. 2 interactors.
IntActiQ3V3R1. 9 interactors.
MINTiMINT-4114903.
STRINGi10090.ENSMUSP00000036178.

PTM databases

iPTMnetiQ3V3R1.
PhosphoSitePlusiQ3V3R1.
SwissPalmiQ3V3R1.

2D gel databases

REPRODUCTION-2DPAGEQ3V3R1.

Proteomic databases

EPDiQ3V3R1.
MaxQBiQ3V3R1.
PaxDbiQ3V3R1.
PeptideAtlasiQ3V3R1.
PRIDEiQ3V3R1.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000043735; ENSMUSP00000036178; ENSMUSG00000040675.
ENSMUST00000117291; ENSMUSP00000112870; ENSMUSG00000040675.
ENSMUST00000120585; ENSMUSP00000112897; ENSMUSG00000040675.
GeneIDi270685.
KEGGimmu:270685.
UCSCiuc007ehj.2. mouse.

Organism-specific databases

CTDi25902.
MGIiMGI:1924836. Mthfd1l.

Phylogenomic databases

eggNOGiKOG4230. Eukaryota.
COG0190. LUCA.
COG2759. LUCA.
GeneTreeiENSGT00860000133777.
HOGENOMiHOG000040280.
HOVERGENiHBG004916.
InParanoidiQ3V3R1.
KOiK13402.
OMAiCSHDFLS.
OrthoDBiEOG091G01BU.
PhylomeDBiQ3V3R1.
TreeFamiTF300623.

Enzyme and pathway databases

UniPathwayiUPA00193.
BRENDAi6.3.4.3. 3474.
ReactomeiR-MMU-196757. Metabolism of folate and pterines.

Miscellaneous databases

ChiTaRSiMthfd1l. mouse.
EvolutionaryTraceiQ3V3R1.
PROiQ3V3R1.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000040675.
GenevisibleiQ3V3R1. MM.

Family and domain databases

CDDicd00477. FTHFS. 1 hit.
Gene3Di3.40.50.300. 2 hits.
3.40.50.720. 2 hits.
HAMAPiMF_01543. FTHFS. 1 hit.
InterProiIPR000559. Formate_THF_ligase.
IPR020628. Formate_THF_ligase_CS.
IPR016040. NAD(P)-bd_dom.
IPR027417. P-loop_NTPase.
IPR000672. THF_DH/CycHdrlase.
IPR020630. THF_DH/CycHdrlase_cat_dom.
IPR020631. THF_DH/CycHdrlase_NAD-bd_dom.
[Graphical view]
PfamiPF01268. FTHFS. 1 hit.
PF00763. THF_DHG_CYH. 1 hit.
PF02882. THF_DHG_CYH_C. 1 hit.
[Graphical view]
PRINTSiPR00085. THFDHDRGNASE.
SUPFAMiSSF51735. SSF51735. 1 hit.
SSF52540. SSF52540. 2 hits.
PROSITEiPS00721. FTHFS_1. 1 hit.
PS00722. FTHFS_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiC1TM_MOUSE
AccessioniPrimary (citable) accession number: Q3V3R1
Secondary accession number(s): Q3TVQ0
, Q3V402, Q80V98, Q8CAL0, Q8K2N5
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 1, 2008
Last sequence update: July 1, 2008
Last modified: November 30, 2016
This is version 99 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  4. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.