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Protein Page:
MTR (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
MTR Catalyzes the transfer of a methyl group from methyl- cobalamin to homocysteine, yielding enzyme-bound cob(I)alamin and methionine. Subsequently, remethylates the cofactor using methyltetrahydrofolate. Defects in MTR are the cause of methylcobalamin deficiency type G (cblG); also known as homocystinuria-megaloblastic anemia complementation type G. It is an autosomal recessive inherited disease that causes mental retardation, macrocytic anemia, and homocystinuria. Mild deficiency in MS activity could be associated with mild hyperhomocysteinemia, a risk factor for cardiovascular disease and possibly neural tube defects. MS mutations could also be involved in tumorigenesis. Defects in MTR may be a cause of susceptibility to folate-sensitive neural tube defects (FS-NTD). The most common NTDs are open spina bifida (myelomeningocele) and anencephaly. Genetic defects in MTR may affect the risk of spina bifida via the maternal rather than the embryonic genotype. Belongs to the vitamin-B12 dependent methionine synthase family. Note: This description may include information from UniProtKB.
Protein type: Cofactor and Vitamin Metabolism - one carbon pool by folate; Amino Acid Metabolism - cysteine and methionine; Methyltransferase; EC 2.1.1.13
Chromosomal Location of Human Ortholog: 1q43
Cellular Component: cytosol
Molecular Function: homocysteine S-methyltransferase activity; zinc ion binding; cobalamin binding; methionine synthase activity
Biological Process: methylation; nervous system development; vitamin metabolic process; cobalamin metabolic process; methionine biosynthetic process; sulfur amino acid metabolic process; xenobiotic metabolic process; pteridine and derivative metabolic process; water-soluble vitamin metabolic process
Reference #:  Q99707 (UniProtKB)
Alt. Names/Synonyms: 5-methyltetrahydrofolate--homocysteine methyltransferase; 5-methyltetrahydrofolate-homocysteine methyltransferase; 5-methyltetrahydrofolate-homocysteine methyltransferase 1; cblG; cobalamin-dependent methionine synthase; FLJ33168; FLJ43216; FLJ45386; METH; Methionine synthase; MS; MTR; Vitamin-B12 dependent methionine synthase
Gene Symbols: MTR
Molecular weight: 140,527 Da
Basal Isoelectric point: 5.39  Predict pI for various phosphorylation states
Select Structure to View Below

MTR

Protein Structure Not Found.


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 1 S2-p ______MsPALQDLS
0 1 K16-ub SQPEGLKktLRDEIN
0 1 T17-p QPEGLKktLRDEINA
0 1 K28-ub EINAILQkRIMVLDG
0 1 T39-p VLDGGMGtMIQREKL
0 1 S121-p AYRMNMCsAGVARKA
0 1 T133-p RKAAEEVtLQTGIKR
0 1 K231-ub ISGTIVDkSGRTLSG
0 1 K339 REIAEAVKNCKPRVP
0 1 Y395-p KLIMAGNyEEALCVA
0 1 Y491-p KARKIKKyGAAMVVM
0 1 Y520-p IRVCTRAyHLLVKKL
0 1 K645-ub KDPEATEkLLRYAQT
0 4 Y677-p PVEERLEyALVKGIE
0 1 K685-ub ALVKGIEkHIIEDTE
0 1 Y701-p ARLNQKKyPRPLNII
0 3 K727-ub GDLFGAGkMFLPQVI
0 1 K735 MFLPQVIKSARVMKK
0 1 T758-p MEKEREEtRVLNGtV
0 1 T764-p EtRVLNGtVEEEDPY
0 1 T774-p EEDPYQGtIVLATVK
0 1 K789-ub GDVHDIGkNIVGVVL
0 2 K817-ub TPCDKILkAALDHkA
0 2 K823-ub LkAALDHkADIIGLS
0 1 R853 EMERLAIRIPLLIGG
0 3 Y905-p DENLKDEyFEEIMEE
0 3 Y913-p FEEIMEEyEDIRQDH
0 1 Y921-p EDIRQDHyESLKERR
0 1 Y929-p ESLKERRyLPLSQAR
0 1 K1000-ub FPKIFNDkTVGGEAR
0 1 Y1010-p GGEARKVyDDAHNML
0 1 K1024-ub LNTLISQkKLRARGV
0 1 S1155-p ELWAYCGsEQLDVAD
0 1 D1194 LTMWRLADIEQSTGI
0 1 S1206-p TGIRLTEsLAMAPAS
0 1 Y1219-p ASAVSGLyFsNLKSK
0 1 S1221-p AVSGLyFsNLKSKYF
0 1 T1264 GPILGYDTD______
  mouse

 
- under review  
K3 _____MKKTLQDEIE
T4 ____MKKTLQDEIEA
K15 EIEAILRKRIMVLDG
T26 VLDGGMGTMIQRYKL
S108 AYRMNKCSADVARKA
T120 RKAAEEITLQTGVKR
K219 ISGTIVDKSGRTLSG
K327-sc REIAEAVkKCKPRVP
Y383 KLIMAGNYEEALSIA
F479 KARKIKKFGAAVVVM
Y508 VNVCTRAYHLLVDKV
K633 KDSEATEKLLRYAQT
Y665 SIEERLEYALVKGIE
K673 ALVKGIEKHIVEDTE
Y689 ARLNGEKYPRPLNII
K715-ub GDLFGAGkMFLPQVI
K723-ub MFLPQVIkSARVMKK
A746 MEKEREEARLINGSV
S752 EARLINGSVEEEDPY
T762 EEDPYQGTIVLATVK
K777 GDVHDIGKNIVGVVL
Q805 TPCDKILQAALDHKA
K811 LQAALDHKADIIGLS
K841-ub EMERLAIkIPLLIGG
Y893 DENLRDDYFEEILEE
Y901 FEEILEEYEDIRQDH
Y909 EDIRQDHYESLKERK
Y917 ESLKERKYVPLSQAR
K988 FPKIFNDKAVGEEAR
Y998 GEEARKVYNDAQNML
K1012 LNILISQKKLQARGV
S1143 ELWAYSRSEQLGVPD
S1182-p LTMWRLAsIEQATGI
S1194 TGIRLTESLAMAPAS
Y1207 ASAVSGLYFSNVKAK
S1209 AVSGLYFSNVKAKYF
T1252-p GPILGYDtD______
  rat

 
- under review  
K3 _____MKKTLQDEIE
T4 ____MKKTLQDEIEA
K15 EIEAILRKRIMVLDG
T26 VLDGGMGTMIQRYKL
S108 AYRMNKCSADVARKA
T120 RKAAEEITLQTGVKR
K219 ISGTIVDKSGRTLSG
K327 REIAEAVKNCKPRVP
Y383 KLIMAGNYEEALSVA
F479 KARKIKKFGAAVVVM
Y508 VSVCTRAYHLLVEKV
K633 RDAEATEKLLRYAQT
Y665 SIEERLEYALVKGIE
K673 ALVKGIEKHIVEDTE
Y689 ARLNREKYPRPLNII
K715 GDLFGAGKMFLPQVI
K723 MFLPQVIKSARVMKK
A746 MEKEREEARVLNGSV
S752 EARVLNGSVEEEDPY
T762 EEDPYQGTIVLATVK
K777 GDVHDIGKNIVGVVL
Q805 TPCDKILQAALDHKA
K811 LQAALDHKADIIGLS
K841 EMERLAIKIPLLIGG
Y893 DENLKDDYFEEILEE
Y901 FEEILEEYEDIRQDH
Y909 EDIRQDHYESLKERK
Y917 ESLKERKYLPLSQAR
K988 FPKIFNDKAVGEEAR
Y998-p GEEARKVyEDAQNML
K1012 LSILISRKKLRARGV
S1143 ELWAYCGSEQLGVTD
N1182 LTMWRLANIEQATGI
S1194 TGIRLTESLAMAPAS
Y1207 ASAVSGLYFSNVKSK
S1209 AVSGLYFSNVKSKYF
T1252-p GPILGYDtD______
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