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Succinyl CoA: 3-Ketoacid CoA Transferase Deficiency
smpdb.ca/view/SMP0000569DiseaseOnce in extrahepatic tissues, SCOT converts AcAc to acetoacetyl‐CoA and T2 cleaves acetoacetyl‐CoA into acetyl‐CoA. … Succinyl CoA: 3-Ketoacid CoA Transferase (SCOT) deficiency is a rare inherited metabolic disorder causing reduction of ketone body utilization.
Enzymes: Acetyl-CoA acetyltransferase, mitochondrial, Hydroxymethylglutaryl-CoA lyase, mitochondrialSuccinyl CoA: 3-Ketoacid CoA Transferase Deficiency
smpdb.ca/view/SMP0125783DiseaseOnce in extrahepatic tissues, SCOT converts AcAc to acetoacetyl‐CoA and T2 cleaves acetoacetyl‐CoA into acetyl‐CoA. … Disease Pathway Succinyl CoA: 3-Ketoacid CoA Transferase (SCOT) deficiency is a rare inherited metabolic disorder causing reduction of ketone body utilization.
Enzymes: Acetyl-CoA acetyltransferase, mitochondrial, Hydroxymethylglutaryl-CoA lyase, mitochondrialPantothenate and CoA Biosynthesis
smpdb.ca/view/SMP0000027MetabolicIn the final step of coenzyme A synthesization, bifunctional coenzyme A synthase catalyzes dephospho-CoA into coenzyme A. … This compound then undergoes two reactions, both resulting in the production of dephospho-CoA; the first reaction uses ectonucleotide pyrophosphatase/phosphodiesterase family member 1, the second uses
Isobutyryl-CoA Dehydrogenase Deficiency
smpdb.ca/view/SMP0125675DiseaseThis enzyme is responsible for the third step in the breakdown of valine and converts isobutyryl-CoA into methylacrylyl-CoA. … Isobutyryl-coenzyme A dehydrogenase is a mitochondrial protein that belongs to the acyl-CoA dehydrogenase family of enzymes.
Enzymes: Enoyl-CoA hydratase, mitochondrial, Methylmalonyl-CoA mutase, mitochondrialSalicylic Acid COX Action Pathway
smpdb.ca/view/SMP0124590Drug actionIt targets the prostaglandin G/H synthase-1 (COX-1) and prostaglandin G/H synthase-2 (COX-2) in the cyclooxygenase pathway. … Salicylic acid inhibits the action of COX-1 and COX-2 on the endoplasmic reticulum membrane. This reduces the formation of prostaglandin H2 and therefore, prostaglandin E2 (PGE2).
Malonyl-CoA Decarboxylase Deficiency
smpdb.ca/view/SMP0000502DiseaseMitochondrial malonyl CoA decarboxylase catalyzes the conversion of intramitochondrial malonyl CoA to acetyl CoA, which is a key product that involve in many biochemical reactions. … malonyl CoA decarboxylase due to reduced activity.
Enzymes: Acetyl-CoA carboxylase 1, Malonyl-CoA decarboxylase, mitochondrialIsobutyryl-CoA Dehydrogenase Deficiency
smpdb.ca/view/SMP0000523DiseaseThis enzyme is responsible for the third step in the breakdown of valine and converts isobutyryl-CoA into methylacrylyl-CoA. … Isobutyryl-coenzyme A dehydrogenase is a mitochondrial protein that belongs to the acyl-CoA dehydrogenase family of enzymes.
Enzymes: Enoyl-CoA hydratase, mitochondrial, Methylmalonyl-CoA mutase, mitochondrialMalonyl-CoA Decarboxylase Deficiency
smpdb.ca/view/SMP0125621DiseaseMitochondrial malonyl CoA decarboxylase catalyzes the conversion of intramitochondrial malonyl CoA to acetyl CoA, which is a key product that involve in many biochemical reactions. … malonyl CoA decarboxylase due to reduced activity.
Enzymes: Acetyl-CoA carboxylase 1, Malonyl-CoA decarboxylase, mitochondrialVery-Long-Chain Acyl-CoA Dehydrogenase Deficiency (VLCAD)
smpdb.ca/view/SMP0000540Diseaselong-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 moreEnzymes: Enoyl-CoA hydratase, mitochondrial, Long-chain-fatty-acid--CoA ligase 1Short-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (HADH)
smpdb.ca/view/SMP0000568DiseaseThis enzyme is responsible for the beta-oxidation of 3-hydroxyhexanoyl-CoA and 3-hydroxybutyryl-CoA into 3-oxohexanoyl-CoA and acetoacetyl-CoA respectively. … 3-hydroxyacyl-CoA dehydrogenase deficiency, also known as HADH deficiency or formerly SCHAD deficiency, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder of the mitochondrial
Enzymes: Enoyl-CoA hydratase, mitochondrial, 3-ketoacyl-CoA thiolase, mitochondrial