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Displaying results 8531 - 8540 of 10000 in total
  1. Pathway

    Acylcarnitine (2S,3R)-3-Hydroxy-2-methylbutanoylcarnitine

    smpdb.ca/view/SMP0123317
    Metabolic

    (2S,3R)-3-Hydroxy-2-methylbutanoylcarnitine is an acylcarnitine. … In forming an acylcarnitine derivative, (2S,3R)-3-hydroxy-2-methylbutanoyl-CoA reacts with L-carnitine to form (2S,3R)-3-hydroxy-2-methylbutanoylcarnitine.

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

    Acylcarnitine 9-(5-pentylfuran-2-yl)nonanoylcarnitine

    smpdb.ca/view/SMP0123527
    Metabolic

    9-(5-pentylfuran-2-yl)nonanoylcarnitine is an acylcarnitine. … Once in the matrix, 9-(5-pentylfuran-2-yl)nonanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 9-(5-pentylfuran-2-

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

    Acylcarnitine (2S,3R)-3-hydroxy-2-methylpentanedioylcarnitine

    smpdb.ca/view/SMP0123829
    Metabolic

    (2S,3R)-3-hydroxy-2-methylpentanedioylcarnitine is an acylcarnitine. … In forming an acylcarnitine derivative, (2S,3R)-3-hydroxy-2-methylpentanedioyl-CoA reacts with L-carnitine to form (2S,3R)-3-hydroxy-2-methylpentanedioylcarnitine.

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

    2-Methyl-3-hydroxybutyryl-CoA Dehydrogenase Deficiency

    smpdb.ca/view/SMP0000137
    Disease

    dehydrogenase type-2. … A deficiency in this enzyme results in accumulation of L-lactic acid in blood, spinal fluid, and urine; 2-ethylhydracrylic acid, 2-methyl-3-hydroxybutyric acid, and tiglylglycine in urine.

    Drugs: Pyridoxal phosphate · Biotin · Succinic acid · Glutamic acid · Pyridoxal · Leucine +10 more
    Enzymes: 3-hydroxyacyl-CoA dehydrogenase type-2, 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
  5. Pathway

    Tyrosinemia Type 2 (or Richner-Hanhart Syndrome)

    smpdb.ca/view/SMP0000369
    Disease

    Tyrosinemia II also known as Richner-Hanhart syndrome is an autosomal recessive disorder caused by a mutation in the TAT gene the encodes for tyrosine aminotransferase. A defect in this enzyme causes excess tyrosine to accumulate in the ...

    Drugs: Pyridoxal phosphate · Phenylalanine · Adenosine phosphate · Tyrosine · Glutamic acid · ATP +11 more
  6. Pathway

    Acylcarnitine (2R,3S)-3-Hydroxy-2-methylbutanoylcarnitine

    smpdb.ca/view/SMP0123320
    Metabolic

    (2R,3S)-3-Hydroxy-2-methylbutanoylcarnitine is an acylcarnitine. … In forming an acylcarnitine derivative, (2R,3S)-3-hydroxy-2-methylbutanoyl-CoA reacts with L-carnitine to form (2R,3S)-3-hydroxy-2-methylbutanoylcarnitine.

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

    Acylcarnitine 7-(5-heptylfuran-2-yl)heptanoylcarnitine

    smpdb.ca/view/SMP0123518
    Metabolic

    Once in the matrix, 7-(5-heptylfuran-2-yl)heptanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 7-(5-heptylfuran-2 … 7-(5-heptylfuran-2-yl)heptanoylcarnitine is an acylcarnitine.

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

    Acylcarnitine 8-(5-butylfuran-2-yl)octanoylcarnitine

    smpdb.ca/view/SMP0123522
    Metabolic

    8-(5-butylfuran-2-yl)octanoylcarnitine is an acylcarnitine. … Once in the matrix, 8-(5-butylfuran-2-yl)octanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 8-(5-butylfuran-2-yl

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

    Acylcarnitine 8-(5-pentylfuran-2-yl)octanoylcarnitine

    smpdb.ca/view/SMP0123523
    Metabolic

    8-(5-pentylfuran-2-yl)octanoylcarnitine is an acylcarnitine. … Once in the matrix, 8-(5-pentylfuran-2-yl)octanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 8-(5-pentylfuran-2-

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

    Acylcarnitine 8-(5-hexylfuran-2-yl)octanoylcarnitine

    smpdb.ca/view/SMP0123524
    Metabolic

    8-(5-hexylfuran-2-yl)octanoylcarnitine is an acylcarnitine. … Once in the matrix, 8-(5-hexylfuran-2-yl)octanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 8-(5-hexylfuran-2-yl

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