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Displaying results 21 - 26 of 26 in total
  1. Pathway

    Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD)

    smpdb.ca/view/SMP0000544
    Disease

    HADHA converts medium- and long-chain 2-enoyl-CoA compounds into the corresponding 3-ketoacyl-CoA compounds when NAD is present, and acetyl-CoA when NAD and CoASH are present. … MCADD is an autosomal recessive disorder associated with a mutation in the enzyme known as hydroxyacyl-CoA dehydrogenase (HADHA).

    Drugs: NADH · Palmitic Acid
    Enzymes: Enoyl-CoA hydratase, mitochondrial, 3-ketoacyl-CoA thiolase, mitochondrial
  2. Pathway

    Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency (VLCAD)

    smpdb.ca/view/SMP0125741
    Disease

    long-chain specific acyl-CoA dehydrogenase. … Very long-chain specific acyl-CoA dehydrogenase breakdown certain fats to energy.

    Drugs: Adenosine phosphate · NADH · ATP · Levocarnitine · Magnesium cation · Flavin adenine dinucleotide +2 more
    Enzymes: Enoyl-CoA hydratase, mitochondrial, Long-chain-fatty-acid--CoA ligase 1
  3. Pathway

    Short-Chain Acyl-CoA Dehydrogenase Deficiency (SCAD Deficiency)

    smpdb.ca/view/SMP0000235
    Disease

    Short Chain Acyl CoA Dehydrogenase Deficiency (SCAD Deficiency) is caused by mutation in the gene encoding short-chain acyl-CoA dehydrogenase, an enzyme which normally breaks down short chain fatty acids

    Drugs: Adenosine phosphate · NADH · ATP · Levocarnitine · Magnesium cation · Flavin adenine dinucleotide +2 more
    Enzymes: Enoyl-CoA hydratase, mitochondrial, Long-chain-fatty-acid--CoA ligase 1
  4. Pathway

    Short-Chain Acyl-CoA Dehydrogenase Deficiency (SCAD Deficiency)

    smpdb.ca/view/SMP0125738
    Disease

    Short Chain Acyl CoA Dehydrogenase Deficiency (SCAD Deficiency) is caused by mutation in the gene encoding short-chain acyl-CoA dehydrogenase, an enzyme which normally breaks down short chain fatty acids

    Drugs: Adenosine phosphate · NADH · ATP · Levocarnitine · Magnesium cation · Flavin adenine dinucleotide +2 more
    Enzymes: Enoyl-CoA hydratase, mitochondrial, Long-chain-fatty-acid--CoA ligase 1
  5. Pathway

    Long Chain Acyl-CoA Dehydrogenase Deficiency (LCAD) (Fatty Acid Metabolism)

    smpdb.ca/view/SMP0125740
    Disease

    Long Chain Acyl CoA Dehydrogenase Deficiency (LCADD) is a rare disorder that is inherited through an autosomal recessive trait, and prevents the body from properly metabolizing very long chain fatty acids

    Drugs: Adenosine phosphate · NADH · ATP · Levocarnitine · Magnesium cation · Flavin adenine dinucleotide +2 more
    Enzymes: Enoyl-CoA hydratase, mitochondrial, Long-chain-fatty-acid--CoA ligase 1
  6. Pathway

    Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase Deficiency (Fatty Acid Elongation in Mitochondria)

    smpdb.ca/view/SMP0125748
    Disease

    HADHA converts medium- and long-chain 2-enoyl-CoA compounds into the corresponding 3-ketoacyl-CoA compounds when NAD is present, and acetyl-CoA when NAD and CoASH are present. … MCADD is an autosomal recessive disorder associated with a mutation in the enzyme known as hydroxyacyl-CoA dehydrogenase (HADHA).

    Drugs: NADH · Palmitic Acid
    Enzymes: Enoyl-CoA hydratase, mitochondrial, 3-ketoacyl-CoA thiolase, mitochondrial