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Displaying results 61 - 70 of 2725 in total
  1. Pathway

    S-Adenosylhomocysteine (SAH) Hydrolase Deficiency

    smpdb.ca/view/SMP0000214
    Disease

    AdoHcyase deficiency causes a buildup of homocysteine which may be then converted into methionine or cysteine. … The accumulation of methionine as a result of AHCY deficiency may lead to signs and symptoms associated with hypermethioninemia, including mental and motor retardation, dysmorphism (unusual facial features

    Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 more
    Enzymes: Methionine synthase, Methionine--tRNA ligase, cytoplasmic
  2. Pathway

    Glycine N-Methyltransferase Deficiency

    smpdb.ca/view/SMP0000222
    Disease

    This disorder is characterized by a large accumulation of methionine in the plasma and transaminases in the serum. Symptoms of the disorder include hepatomegaly. … Glycine N-methyltransferase deficiency, also called GNMT deficiency, is an autosomal recessive disorder of methionine metabolism caused by a defective glycine N-methyltransferase (GNMT).

    Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 more
    Enzymes: Methionine synthase, Methionine--tRNA ligase, cytoplasmic
  3. Pathway

    Homocystinuria-Megaloblastic Anemia Due to Defect in Cobalamin Metabolism, cblG Complementation Type

    smpdb.ca/view/SMP0000570
    Disease

    Methionine synthase deficiency is characterized by an increase in homocysteine levels in the body and excreted in the urine, as well as decreased levels of methionine in the blood. … It is caused by a mutation in the MTR gene which encodes the enzyme methionine synthase.

    Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 more
    Enzymes: Methionine synthase, Methionine--tRNA ligase, cytoplasmic
  4. Pathway

    Glycine N-Methyltransferase Deficiency

    smpdb.ca/view/SMP0125681
    Disease

    This disorder is characterized by a large accumulation of methionine in the plasma and transaminases in the serum. Symptoms of the disorder include hepatomegaly. … Glycine N-methyltransferase deficiency, also called GNMT deficiency, is an autosomal recessive disorder of methionine metabolism caused by a defective glycine N-methyltransferase (GNMT).

    Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 more
    Enzymes: Methionine synthase, Methionine--tRNA ligase, cytoplasmic
  5. Drug

    Cyanocobalamin

    go.drugbank.com/drugs/DB00115
    ApprovedInvestigationalNutraceutical

    Cyanocobalamin (commonly known as Vitamin B12) is a highly complex, essential vitamin, owing its name to the fact that it contains the mineral, cobalt. This vitamin is produced naturally by bacteria , and is necessary for DNA synthesis a...

  6. Pathway

    Hypermethioninemia

    smpdb.ca/view/SMP0125679
    Disease

    This outputted compound through the its respective pathway may be turned back into cysteine methionine.

    Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 more
    Enzymes: Methionine synthase, Methionine-R-sulfoxide reductase B3
  7. Drug

    Hydroxocobalamin

    go.drugbank.com/drugs/DB00200
    ApprovedInvestigational

    Hydroxocobalamin, also known as vitamin B12a and hydroxycobalamin, is an injectable form of vitamin B 12 that has been used therapeutically to treat vitamin B 12 deficiency. It is also used in cyanide poisoning, Leber's optic atrophy, an...

  8. Pathway

    Cystathionine beta-Synthase Deficiency

    smpdb.ca/view/SMP0125678
    Disease

    Treatment includes folic acid, vitamin B6, vitamin B12, and a methionine-restricted diet. … A deficiency in this enzyme results in accumulation of L-cystathionine, homocysteine, and L-homocystine in plasma and urine; and L-methionine and ornithine in plasma.

    Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 more
    Enzymes: Methionine synthase, Methionine-R-sulfoxide reductase B3
  9. Pathway

    Methylenetetrahydrofolate Reductase Deficiency (MTHFRD)

    smpdb.ca/view/SMP0125682
    Disease

    A defect in this enzyme results in accumulation of homocysteine and methionine in both plasma and urine. … Methylenetetrahydrofolate reductase catalyzes the conversion of 5,10-methylenetetrahydrofolate to 5-methyltetrahydrofolate, a co-substrate for homocysteine remethylation to methionine.

    Drugs: Pyridoxal phosphate · Tetrahydrofolic acid · Ademetionine · Choline · Adenosine phosphate · Serine +17 more
    Enzymes: Methionine synthase, Methionine-R-sulfoxide reductase B3
  10. Pathway

    Betaine Metabolism

    smpdb.ca/view/SMP0000123
    Metabolic

    In the liver, betaine functions as a methyl donor similar to choline, folic acid, S-adenosyl methionine and vitamin B12.

    Drugs: Tetrahydrofolic acid · Ademetionine · Choline · Methionine · NADH · ATP +8 more
    Enzymes: Methionine synthase, Methionine adenosyltransferase 2 subunit beta