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Fatty Acid Biosynthesis
smpdb.ca/view/SMP0000456MetabolicThe biosynthesis of fatty acids primarily occurs in liver and lactating mammary glands. … This saturated acyl moiety is then transfer back to the active site of the beta-ketoacyl synthase domain, another molonyl-CoA is loaded and the process repeats.
Drugs: Biotin · Acetoacetic acid · Butenoic Acid · Malonic acid · (R)-3-hydroxytetradecanoic acid · Lauric acid +8 moreEnzymes: Fatty acid synthaseFatty Acid Elongation in Mitochondria
smpdb.ca/view/SMP0000054MetabolicThere are 3 major processes that modify palmitic acid: elongation, desaturation and hydroxylation. … In particular, both pathways make use of acetyl-CoA acyltransferase, 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase.
Enzymes: 3-ketoacyl-CoA thiolase, mitochondrial, 3-hydroxyacyl-CoA dehydrogenase type-2Fatty Acid Metabolism
smpdb.ca/view/SMP0000051MetabolicFatty acids constitute a large energy source for the body. The cellular membrane is also made up of fatty acids. During starvation times, fatty acids can provide energy to humans for numerous days. … Fatty acid metabolism is also known as beta-oxidation. During metabolism, acetyl CoA is produced that can then enter the citric acid cycle.
Drugs: Adenosine phosphate · NADH · ATP · Levocarnitine · Magnesium cation · Flavin adenine dinucleotide +2 moreEnzymes: Long-chain-fatty-acid--CoA ligase 1, 3-ketoacyl-CoA thiolase, mitochondrialLong-Chain-3-Hydroxyacyl-CoA Dehydrogenase Deficiency (Fatty Acid Elongation in Mitochondria)
smpdb.ca/view/SMP0125748DiseaseHADHA catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. … 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.
Enzymes: 3-ketoacyl-CoA thiolase, mitochondrial, 3-hydroxyacyl-CoA dehydrogenase type-2Glutaric Aciduria Type I (Fatty Acid Metabolism)
smpdb.ca/view/SMP0125737DiseaseA deficiency in this enzyme results in accumulation of 3-hydroxybutyric acid, 3-hydroxyglutaric acid, glutaconic acid, glutaric acid, and ketone bodies in urine. … Symptoms include encephalopathy, grimacing, dystonia, metabolic acidosis, and hygroma. Treatment includes a low-protein diet, L-carnitine, riboflavin, and anticonvulsants.
Drugs: Adenosine phosphate · NADH · ATP · Levocarnitine · Magnesium cation · Flavin adenine dinucleotide +4 moreEnzymes: Long-chain-fatty-acid--CoA ligase 1, Solute carrier family 13 member 3Long Chain Acyl-CoA Dehydrogenase Deficiency (LCAD) (Fatty Acid Metabolism)
smpdb.ca/view/SMP0125740DiseaseLong 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 … This disorder occurs in the mitochondria, where the metabolization of fatty acids takes place. Early-onset LCADD patients usually begin to exhibit symptoms just days or weeks after birth.
Drugs: Adenosine phosphate · NADH · ATP · Levocarnitine · Magnesium cation · Flavin adenine dinucleotide +2 moreEnzymes: Long-chain-fatty-acid--CoA ligase 1, 3-ketoacyl-CoA thiolase, mitochondrial3-Hydroxyisobutyric Acid Dehydrogenase Deficiency
smpdb.ca/view/SMP0125673DiseaseThis enzyme converts 2-methyl-3-oxopropanoate, CoA and water into propanoyl-CoA, using NAD+ as an oxidizing agent, and producing a hydrogen ion and hydrogencarbonate as byproducts. … 3-Hydroxyisobutyric acid dehydrogenase deficiency (3-hydroxyisobutyric aciduria) is an extremely rare inborn error of metabolism (IEM), potentially caused by numerous mechanisms.
Enzymes: 3-ketoacyl-CoA thiolase, mitochondrial, 3-hydroxyacyl-CoA dehydrogenase type-23-Hydroxyisobutyric Acid Dehydrogenase Deficiency
smpdb.ca/view/SMP0000521DiseaseThis enzyme converts 2-methyl-3-oxopropanoate, CoA and water into propanoyl-CoA, using NAD+ as an oxidizing agent, and producing a hydrogen ion and hydrogencarbonate as byproducts. … 3-Hydroxyisobutyric acid dehydrogenase deficiency (3-hydroxyisobutyric aciduria) is an extremely rare inborn error of metabolism (IEM), potentially caused by numerous mechanisms.
Enzymes: 3-ketoacyl-CoA thiolase, mitochondrial, 3-hydroxyacyl-CoA dehydrogenase type-23-beta-Hydroxysteroid Dehydrogenase Deficiency
smpdb.ca/view/SMP0000718Disease3-beta-hydroxysteroid dehydrogenase (HSD) deficiency is an extremely rare inborn error of metabolism (IEM) and autosomal recessive disorder of the steroidogenesis pathway. … When the enzyme is not correctly produced, cortisol levels in the cell are lowered, and as cortisol is used in the production of other steroids, it may affect their levels as well. 3-beta-HSD deficiency
Enzymes: 3-oxo-5-beta-steroid 4-dehydrogenase, 3 beta-hydroxysteroid dehydrogenase/Delta 5-->4-isomerase type 13-beta-Hydroxysteroid Dehydrogenase Deficiency
smpdb.ca/view/SMP0125804Disease3-beta-hydroxysteroid dehydrogenase (HSD) deficiency is an extremely rare inborn error of metabolism (IEM) and autosomal recessive disorder of the steroidogenesis pathway. … When the enzyme is not correctly produced, cortisol levels in the cell are lowered, and as cortisol is used in the production of other steroids, it may affect their levels as well. 3-beta-HSD deficiency
Enzymes: 3-oxo-5-beta-steroid 4-dehydrogenase, 3 beta-hydroxysteroid dehydrogenase/Delta 5-->4-isomerase type 1