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Displaying results 101 - 110 of 135872 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. … Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency, which is also known LCHADD, is a rare inherited inborn error of metabolism (IEM) of long-chain fatty acid metabolism.

    Drugs: NADH · Palmitic Acid
  2. Pathway

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

    smpdb.ca/view/SMP0125741
    Disease

    Very long-chain acyl-CoA dehydrogenase deficiency (VLCAD), also called ACADL and VLCAD, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder, which is caused by a defective very … long-chain specific acyl-CoA dehydrogenase.

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

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

    smpdb.ca/view/SMP0000540
    Disease

    Very long-chain acyl-CoA dehydrogenase deficiency (VLCAD), also called ACADL and VLCAD, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder, which is caused by a defective very … long-chain specific acyl-CoA dehydrogenase.

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

    Short-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (HADH)

    smpdb.ca/view/SMP0000568
    Disease

    beta-oxidation of short chain saturated fatty acid pathway. … This enzyme is responsible for the beta-oxidation of 3-hydroxyhexanoyl-CoA and 3-hydroxybutyryl-CoA into 3-oxohexanoyl-CoA and acetoacetyl-CoA respectively.

    Drugs: Adenosine phosphate · NADH · ATP · Flavin adenine dinucleotide · Pyrophosphoric acid
    Enzymes: Short-chain specific acyl-CoA dehydrogenase, mitochondrial, Long-chain specific acyl-CoA dehydrogenase, mitochondrial
  5. Pathway

    Short-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (HADH)

    smpdb.ca/view/SMP0125810
    Disease

    beta-oxidation of short chain saturated fatty acid pathway. … This enzyme is responsible for the beta-oxidation of 3-hydroxyhexanoyl-CoA and 3-hydroxybutyryl-CoA into 3-oxohexanoyl-CoA and acetoacetyl-CoA respectively.

    Drugs: Adenosine phosphate · NADH · ATP · Flavin adenine dinucleotide · Pyrophosphoric acid
    Enzymes: Short-chain specific acyl-CoA dehydrogenase, mitochondrial, Long-chain specific acyl-CoA dehydrogenase, mitochondrial
  6. Pathway

    Oxidation of Branched-Chain Fatty Acids

    smpdb.ca/view/SMP0000030
    Metabolic

    After beta-oxidation in the cycle of reactions, an acetyl-CoA unit is released and is recycled into the cycle of reactions in the mitochondria, until the chain is fully broken down into acetyl-CoA, and … Each time the cycle of these reactions is completed, the chain is relieved of two carbons, which are oxidized and are taken away by NADH and FADH2, energy carriers that collect the carbons energy.

    Drugs: Ascorbic acid · Succinic acid · ATP · Levocarnitine · Magnesium cation · Iron +2 more
    Enzymes: Long-chain-fatty-acid--CoA ligase 1
  7. Pathway

    Mitochondrial Beta-Oxidation of Long Chain Saturated Fatty Acids

    smpdb.ca/view/SMP0000482
    Metabolic

    beta-oxidized as medium chain saturated fatty acids. … These include signalling roles, energy creation roles and enzyme regulation.

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

    Long Chain Acyl-CoA Dehydrogenase Deficiency (LCAD)

    smpdb.ca/view/SMP0000539
    Disease

    Very Long Chain Acyl CoA Dehydrogenase Deficiency (VLCADD) is a rare disorder that is inherited through an autosomal recessive trait, and prevents the body from properly metabolizing very long chain fatty … To help control acute episodes, treatment includes maintaining a high carbohydrate and low fat diet, and avoiding fasting for more than 12 hours.

    Drugs: Adenosine phosphate · NADH · ATP · Levocarnitine · Magnesium cation · Flavin adenine dinucleotide +2 more
    Enzymes: Medium-chain specific acyl-CoA dehydrogenase, mitochondrial, Long-chain-fatty-acid--CoA ligase 1
  9. Pathway

    Beta Oxidation of Very Long Chain Fatty Acids

    smpdb.ca/view/SMP0000052
    Metabolic

    With each turn of the cycle two carbons are removed from the fatty acid chain and the energy of the chemical bonds once housed by the molecule is captured by the reduced energy carriers NADH and FADH2. … The reason the beta-oxidation cycle is found to occur in both mitochondria and peroxisomes in mammals is thought to be that extremely long chain fatty acids will in fact undergo oxidation in both locations

    Drugs: ATP · Levocarnitine · Magnesium cation · Lauric acid
    Enzymes: Long-chain-fatty-acid--CoA ligase 1
  10. 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 … Symptoms include hypoglycemia, hypotonia, microcephaly, failure to thrive, lactic acidosis, peripheral neuropathy, and vomiting.

    Drugs: Adenosine phosphate · NADH · ATP · Levocarnitine · Magnesium cation · Flavin adenine dinucleotide +2 more
    Enzymes: Medium-chain specific acyl-CoA dehydrogenase, mitochondrial, Long-chain-fatty-acid--CoA ligase 1