Search results for MTR

Showing 20 results out of 51

×

Species

Types

Compartments

Reaction types

Search properties

Species

Types

Compartments

Reaction types

Search properties

Protein (3 results from a total of 12)

MTR

Identifier: R-HSA-174376
Species: Homo sapiens
Compartment: cytosol
Primary external reference: UniProt: MTR: Q99707
Identifier: R-HSA-3321942
Species: Homo sapiens
Compartment: cytosol
Primary external reference: UniProt: MTR: Q99707
Identifier: R-HSA-3321914
Species: Homo sapiens
Compartment: cytosol
Primary external reference: UniProt: MTR: Q99707

Set (3 results from a total of 3)

Identifier: R-HSA-3321946
Species: Homo sapiens
Compartment: cytosol
Identifier: R-HSA-3321956
Species: Homo sapiens
Compartment: cytosol
Identifier: R-HSA-5432633
Species: Homo sapiens
Compartment: mitochondrial matrix
Both MTRF1L and ICT1 can bind a standard stop codon in the A-site of the ribosome and cause release of the polypeptide. ICT1 can also cause release of ribosomes stalled in non-standard conformations (e.g. non-standard stop codons, mRNA lacking a stop codon) (inferred from pig mitoribosomes in Akabane et al. 2014).

Pathway (3 results from a total of 3)

Identifier: R-HSA-3359469
Species: Homo sapiens
Defects in MTR cause methylcobalamin deficiency type G (cblG; MIM:250940), an autosomal recessive inherited disease that causes mental retardation, macrocytic anemia, and homocystinuria (Leclerc et al. 1996, Gulati et al. 1996, Watkins et al. 2002).
Identifier: R-HSA-3359467
Species: Homo sapiens
Defects in MTRR cause methylcobalamin deficiency type E (cblE; methionine synthase reductase deficiency; MIM:236270) (Wilson et al. 1999). Patients with cblE exhibit megaloblastic anemia and hyperhomocysteinemia. SAM is used as a methyl donor in many biological reactions and demethylation of SAM produces S-adenosylhomocysteine, which is deadenosylated to form homocysteine. Homocysteine remethylation is carried out by MTR, which requires MTRR to maintain enzyme-bound cobalamin (Cbl) in its active form; but in cblE patients, MTR becomes inactivated and thus homocysteine accumulates.
Identifier: R-HSA-3296469
Species: Homo sapiens
Cobalamin (Cbl, vitamin B12) is a nutrient essential for normal functioning of the brain and nervous system and for the formation of blood. Cbl-dependent methionine synthase (MTR) is required for conversion of 5-methyltetrahydrofolate (metTHF) to tetrahydrofolate (THF), in addition to its role in conversion of homocysteine to methionine. In Cbl deficiency, and in inborn errors of Cbl metabolism that affect function of methionine synthase, inability to regenerate THF from metTHF results in decreased function of folate-dependent reactions that are involved in 2 steps of purine biosynthesis and thymidylate synthesis. Cbl deficiency results in hyperhomocysteinemia (due to defects in the conversion of homocysteine to methionine which requires Cbl as a cofactor) and increased levels of methylmalonic acid (MMA). Methionine is used in myelin production, protein, neurotransmitter, fatty acid and phospholipid production and DNA methylation. Symptoms of Cbl deficiency are bone marrow promegaloblastosis (megaloblastic anemia) due to the inhibition of DNA synthesis (specifically purines and thymidine) and neurological symptoms. The defective genes involved in Cbl deficiencies are described below (Froese & Gravel 2010, Nielsen et al. 2012, Whitehead 2006, Watkins & Rosenblatt 2011, Fowler 1998).

Reaction (3 results from a total of 12)

Identifier: R-HSA-174374
Species: Homo sapiens
Compartment: cytosol
Methionine synthase (MTR) mediates the continuous shuttling of cobalamin (Cbl) between two forms, cob(I)alamin and MeCbl. In this half reaction, the methyl group from MeCbl is transferred to homocysteine (HYCS) to form methionine and regenerate cob(I)alamin (Hall et al. 2000; Leclerc et al. 1996).
Identifier: R-HSA-3149539
Species: Homo sapiens
Compartment: cytosol
Methionine synthase (MTR) mediates the continuous shuttling of cobalamin (Cbl) between two forms, cob(I)alamin and MeCbl. In this half reaction, the methyl group from 5 methyltetrahydrofolate (5-methyl-THF) is transferred to enzyme bound cob(I)alamin to form MeCbl (Hall et al. 2000). Defects in MTR cause methylcobalamin deficiency type G (cblG, methionine synthase deficiency; MIM:250940), an autosomal recessive inherited disease that causes mental retardation, macrocytic anemia, and homocystinuria (Leclerc et al. 1996).
Identifier: R-HSA-3322140
Species: Homo sapiens
Compartment: cytosol
Methionine synthase (MTR) catalyses the transfer of a methyl group from 5-methyltetrahydrofolate (MTHF) to MTR-bound cob(I)alamin to form methylcobalamin (MeCbl). In the second step, the methyl group from MeCbl is transferred to homocysteine (HCYS), forming methionine (L-Met) (Leclerc et al. 1996). Under normal conditions, the enzyme-bound cobalamin shuttles between the cob(I)alamin and MeCbl forms. Every few hundred cycles, enzyme-bound cob(I)alamin is spontaneously oxidized to cob(II)alamin, inactivating MTR. Active MTR is regenerated by MTRR which, in conjunction with MTR, catalyzes reductive methylation of cob(II)alamin to MeCbl using SAM as a methyl donor.

Defects in MTR cause methylcobalamin deficiency type G (cblG, methionine synthase deficiency; MIM:250940), an autosomal recessive inherited disease that causes mental retardation, macrocytic anemia, and homocystinuria. Mutations causing cblG include P1173L, Ile881, H920D, R585*, E1204* and A1204P (Leclerc et al. 1996, Gulati et al. 1996, Watkins et al. 2002).

Complex (3 results from a total of 16)

Identifier: R-HSA-9013305
Species: Homo sapiens
Compartment: cytosol
Identifier: R-HSA-9013303
Species: Homo sapiens
Compartment: cytosol
Identifier: R-HSA-3204322
Species: Homo sapiens
Compartment: cytosol

Interactor (1 results from a total of 1)

Identifier: Q13585
Species: Homo sapiens
Primary external reference: UniProt: Q13585

Chemical Compound (2 results from a total of 2)

Identifier: R-ALL-1237165
Compartment: cytosol
Primary external reference: ChEBI: S-methyl-5-thio-alpha-D-ribose 1-phosphate(2-): 58533
Identifier: R-ALL-1237107
Compartment: cytosol
Primary external reference: ChEBI: S-methyl-5-thio-D-ribulose 1-phosphate(2-): 58548

Icon (2 results from a total of 2)

MTR

Species: Homo sapiens
Curator: Bijay Jassal
Designer: Cristoffer Sevilla
MTR icon
Methionine synthase.
Species: Homo sapiens
Curator: Bijay Jassal
Designer: Cristoffer Sevilla
MTRR icon
Methionine synthase reductase.
Cite Us!