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Displaying results 1 - 10 of 10000 in total
  1. Pathway

    Acylcarnitine (2S)-2-hydroxy-2-(propan-2-yl)butanedioylcarnitine

    smpdb.ca/view/SMP0123461
    Metabolic

    (2S)-2-hydroxy-2-(propan-2-yl)butanedioylcarnitine is an acylcarnitine. … In forming an acylcarnitine derivative, (2S)-2-hydroxy-2-(propan-2-yl)butanedioyl-CoA reacts with L-carnitine to form (2S)-2-hydroxy-2-(propan-2-yl)butanedioylcarnitine.

    Drugs: Biotin · Adenosine phosphate · ATP · Levocarnitine · Magnesium cation · Pyrophosphoric acid +1 more
    Enzymes: Carnitine O-palmitoyltransferase 2, mitochondrial
  2. Pathway

    Acylcarnitine (2Z)-2-(propan-2-yl)but-2-enedioylcarnitine

    smpdb.ca/view/SMP0123554
    Metabolic

    (2Z)-2-(propan-2-yl)but-2-enedioylcarnitine is an acylcarnitine. … In forming an acylcarnitine derivative, (2Z)-2-(propan-2-yl)but-2-enedioyl-CoA reacts with L-carnitine to form (2Z)-2-(propan-2-yl)but-2-enedioylcarnitine.

    Drugs: Biotin · Adenosine phosphate · ATP · Levocarnitine · Magnesium cation · Pyrophosphoric acid +1 more
    Enzymes: Carnitine O-palmitoyltransferase 2, mitochondrial
  3. Pathway

    2-Aminoadipic 2-Oxoadipic Aciduria

    smpdb.ca/view/SMP0000719
    Disease

    2-Aminoadipic 2-oxoadipic aciduria is a disorder of lysine metabolism caused by a defective DHTKD1 gene. … This disease is characterized by a large accumulation of 2-oxoadipate and 2-hydroxyadipate in the urine.

    Drugs: Pyridoxal phosphate · Lysine · Glutamic acid · NADH · Oxogluric acid · Flavin adenine dinucleotide +1 more
    Enzymes: Mitochondrial ornithine transporter 2, Low affinity cationic amino acid transporter 2
  4. Pathway

    Acylcarnitine 2-Ethyl-2-hydroxybutanedioylcarnitine

    smpdb.ca/view/SMP0123462
    Metabolic

    2-Ethyl-2-hydroxybutanedioylcarnitine is an acylcarnitine. … In forming an acylcarnitine derivative, 2-Ethyl-2-hydroxybutanedioyl-CoA reacts with L-carnitine to form 2-Ethyl-2-hydroxybutanedioylcarnitine.

    Drugs: Biotin · Adenosine phosphate · ATP · Levocarnitine · Magnesium cation · Pyrophosphoric acid +1 more
    Enzymes: Carnitine O-palmitoyltransferase 2, mitochondrial
  5. Pathway

    Acylcarnitine 2-Hydroxyundec-2-enoylcarnitine

    smpdb.ca/view/SMP0123963
    Metabolic

    2-Hydroxyundec-2-enoylcarnitine is an acylcarnitine. … Once in the matrix, 2-hydroxyundec-2-enoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 2-hydroxyundec-2-enoyl-CoA

    Drugs: Biotin · Adenosine phosphate · ATP · Levocarnitine · Magnesium cation · Pyrophosphoric acid +1 more
    Enzymes: Carnitine O-palmitoyltransferase 2, mitochondrial
  6. Pathway

    2-Aminoadipic 2-Oxoadipic Aciduria

    smpdb.ca/view/SMP0125703
    Disease

    2-Aminoadipic 2-oxoadipic aciduria is a disorder of lysine metabolism caused by a defective DHTKD1 gene. … This disease is characterized by a large accumulation of 2-oxoadipate and 2-hydroxyadipate in the urine.

    Drugs: Pyridoxal phosphate · Lysine · Glutamic acid · NADH · Oxogluric acid · Flavin adenine dinucleotide +1 more
    Enzymes: Mitochondrial ornithine transporter 2, Low affinity cationic amino acid transporter 2
  7. Pathway

    Acylcarnitine (2E)-2-propylpent-2-enoylcarnitine

    smpdb.ca/view/SMP0123566
    Metabolic

    (2E)-2-propylpent-2-enoylcarnitine is an acylcarnitine. … In forming an acylcarnitine derivative, (2E)-2-propylpent-2-enoyl-CoA reacts with L-carnitine to form (2E)-2-propylpent-2-enoylcarnitine.

    Drugs: Biotin · Adenosine phosphate · ATP · Levocarnitine · Magnesium cation · Pyrophosphoric acid +1 more
    Enzymes: Carnitine O-palmitoyltransferase 2, mitochondrial
  8. Pathway

    Cardiolipin Biosynthesis CL(20:2(11Z,14Z)/18:2(9Z,12Z)/18:2(9Z,12Z)/18:2(9Z,12Z))

    smpdb.ca/view/SMP0029569
    Metabolic

    Third, the enzyme 1-acyl-sn-glycerol-3-phosphate acyltransferase converts LPA into phosphatidic acid (PA or 1,2-diacyl-sn-glycerol 3-phosphate) by esterifying an acyl-group to the sn-2 position of the

    Drugs: NADH · Cytidine-5'-Triphosphate · sn-glycerol 3-phosphate · Pyrophosphoric acid · Dihydroxyacetone phosphate
    Enzymes: Phosphatidate cytidylyltransferase 2
  9. Pathway

    Cardiolipin Biosynthesis CL(18:2(9Z,11Z)/18:2(9Z,12Z)/18:2(9Z,12Z)/18:2(9Z,11Z))

    smpdb.ca/view/SMP0216581
    Metabolic

    Third, the enzyme 1-acyl-sn-glycerol-3-phosphate acyltransferase converts LPA into phosphatidic acid (PA or 1,2-diacyl-sn-glycerol 3-phosphate) by esterifying an acyl-group to the sn-2 position of the

    Drugs: NADH · sn-glycerol 3-phosphate · Dihydroxyacetone phosphate
  10. Pathway

    Cardiolipin Biosynthesis CL(18:2(9Z,11Z)/18:2(9Z,12Z)/18:2(9Z,12Z)/18:2(9Z,12Z))

    smpdb.ca/view/SMP0216582
    Metabolic

    Third, the enzyme 1-acyl-sn-glycerol-3-phosphate acyltransferase converts LPA into phosphatidic acid (PA or 1,2-diacyl-sn-glycerol 3-phosphate) by esterifying an acyl-group to the sn-2 position of the

    Drugs: NADH · sn-glycerol 3-phosphate · Dihydroxyacetone phosphate