Search
Pyruvate Dehydrogenase Complex Deficiency
smpdb.ca/view/SMP0125772DiseasePyruvate dehydrogenase complex deficiency results from a mutation in the E1-alpha polypeptide gene (PDHA1). … PDHA1 encodes the pyruvate dehydrogenase complex (PDC) a critical complex that converts pyruvate from glycolysis to acetyl CoA for the citric acid cycle.
Enzymes: Acyl-coenzyme A thioesterase 12, L-lactate dehydrogenase A-like 6APyruvate Dehydrogenase Complex Deficiency
smpdb.ca/view/SMP0000212DiseasePyruvate dehydrogenase complex deficiency results from a mutation in the E1-alpha polypeptide gene (PDHA1). … PDHA1 encodes the pyruvate dehydrogenase complex (PDC) a critical complex that converts pyruvate from glycolysis to acetyl CoA for the citric acid cycle.
Enzymes: Acyl-coenzyme A thioesterase 12, L-lactate dehydrogenase A-like 6AMitochondrial Complex II Deficiency
smpdb.ca/view/SMP0125754DiseaseBecause complex II is found in the mitochondria, CII deficiency is technically considered a mitochondrial disease. … Mitochondrial complex II deficiency, which is also known as CII deficiency, is a rare form of an inherited inborn error of metabolism (IEM).
Enzymes: Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial, Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial2-Ketoglutarate Dehydrogenase Complex Deficiency
smpdb.ca/view/SMP0000549Disease2-Ketoglutarate dehydrogenase complex deficiency, also known as alpha-ketoglutarate dehydrogenase deficiency or oxoglutaric aciduria, is an autosomal recessive disorder of the Krebs cycle caused by a defective … oxoglutarate dehydrogenase complex (OGDC).
Enzymes: Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial, Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrialMitochondrial Complex II Deficiency
smpdb.ca/view/SMP0000548DiseaseBecause complex II is found in the mitochondria, CII deficiency is technically considered a mitochondrial disease. … Mitochondrial complex II deficiency, which is also known as CII deficiency, is a rare form of an inherited inborn error of metabolism (IEM).
Enzymes: Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial, Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrialCopper gluconate Drug Metabolism
smpdb.ca/view/SMP0130548MetabolicCopper gluconate is a drug that is not metabolized by the human body as determined by current research and biotransformer analysis. … Copper gluconate passes through the liver and is then excreted from the body mainly through the kidney.
Drugs: Copper gluconate2-Ketoglutarate Dehydrogenase Complex Deficiency
smpdb.ca/view/SMP0125755Disease2-Ketoglutarate dehydrogenase complex deficiency, also known as alpha-ketoglutarate dehydrogenase deficiency or oxoglutaric aciduria, is an autosomal recessive disorder of the Krebs cycle caused by a defective … oxoglutarate dehydrogenase complex (OGDC).
Enzymes: Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial, Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrialUndecoylium chloride iodine complex Drug Metabolism
smpdb.ca/view/SMP0130610MetabolicUndecoylium chloride iodine complex is a drug that is not metabolized by the human body as determined by current research and biotransformer analysis. … Undecoylium chloride iodine complex passes through the liver and is then excreted from the body mainly through the kidney.
Tetrakis(2-methoxyisobutylisocyanide)copper(I) tetrafluoroborate Drug Metabolism
smpdb.ca/view/SMP0130603MetabolicTetrakis(2-methoxyisobutylisocyanide)copper(I) tetrafluoroborate is a drug that is not metabolized by the human body as determined by current research and biotransformer analysis. … Tetrakis(2-methoxyisobutylisocyanide)copper(I) tetrafluoroborate passes through the liver and is then excreted from the body mainly through the kidney.
Cardiolipin Biosynthesis CL(a-15:0/a-15:0/a-15:0/a-15:0)
smpdb.ca/view/SMP0361372MetabolicLast, cardiolipin synthase catalyzes the synthesis of cardiolipin by transferring a phosphatidyl group from a second CDP-diacylglycerol to PG. It requires a divalent metal cation cofactor.