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Long Chain Acyl-CoA Dehydrogenase Deficiency (LCAD) (Fatty Acid Metabolism)
smpdb.ca/view/SMP0125740DiseaseTo help control acute episodes, treatment includes maintaining a high carbohydrate and low fat diet, and avoiding fasting for more than 12 hours. … Patients will also be at risk for hypertrophic cardiomyopathy and other heart conditions from age two months to two years.
Drugs: Adenosine phosphate · NADH · ATP · Levocarnitine · Magnesium cation · Flavin adenine dinucleotide +2 moreEnzymes: Long-chain-fatty-acid--CoA ligase 1Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase Deficiency (Fatty Acid Elongation in Mitochondria)
smpdb.ca/view/SMP0125748DiseaseLong-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. … LCHAD-deficiency individuals are also at risk for breathing difficulties, serious heart problems, coma, and sudden death. Fasting or illnesses (e.g. viral infections) can trigger related problems.
Oxidation of Branched-Chain Fatty Acids
smpdb.ca/view/SMP0000030MetabolicNow moving to the mitochondria, there are four reactions that occur, and are repeated for each molecule of the fatty acid. … Unfortunately, traditional fatty acid oxidation does not work for branched-chain fatty acids, or fatty acids that do not have an even number of carbons, like the fatty acid phytanic acid, found in animal
Enzymes: Long-chain-fatty-acid--CoA ligase 1Beta Oxidation of Very Long Chain Fatty Acids
smpdb.ca/view/SMP0000052MetabolicWith 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 oxidation of fatty acid chains is a process of progress through repetition.
Enzymes: Long-chain-fatty-acid--CoA ligase 1Mitochondrial Beta-Oxidation of Long Chain Saturated Fatty Acids
smpdb.ca/view/SMP0000482Metabolicthe citric acid cycle) and uses trifunctional enzyme subunits alpha and beta to create palmityl-CoA. … Fatty acids and their CoA byproducts can be found in many places in the body, playing major roles in many basic functions of the body.
Drugs: Adenosine phosphate · NADH · ATP · Levocarnitine · Magnesium cation · Flavin adenine dinucleotide +2 moreEnzymes: Long-chain-fatty-acid--CoA ligase 1Mitochondrial Beta-Oxidation of Short Chain Saturated Fatty Acids
smpdb.ca/view/SMP0000480MetabolicBeta-oxidation is the major degradative pathway for fatty acid esters in humans. … molecules for hormones, and signalling molecules.
Mitochondrial Beta-Oxidation of Medium Chain Saturated Fatty Acids
smpdb.ca/view/SMP0000481MetabolicBeta-oxidation is the major degradative pathway for fatty acid esters in humans. … molecules for hormones, and signalling molecules.
Drugs: Adenosine phosphate · NADH · ATP · Magnesium cation · Lauric acid · Flavin adenine dinucleotide +1 moreFructosuria
smpdb.ca/view/SMP0000561DiseaseFructosuria, full name essential fructosuria, is a condition that presents no symptoms and is benign. … It is identified by the presence of fructose in the urine, which in people without the condition should not be present.
Enzymes: Fructose-1,6-bisphosphatase 1, Fructose-bisphosphate aldolase AFructosuria
smpdb.ca/view/SMP0125775DiseaseFructosuria, full name essential fructosuria, is a condition that presents no symptoms and is benign. … It is identified by the presence of fructose in the urine, which in people without the condition should not be present.
Enzymes: Fructose-1,6-bisphosphatase 1, Fructose-bisphosphate aldolase APentose Phosphate Pathway
smpdb.ca/view/SMP0000031Metabolic4-phosphate and fructose 6-phosphate; and reaction 8 where transkelotase rearranges of xylulose 5-phosphate and erythrose 4-phosphate to form glyceraldehyde 3-phosphate and fructose-6-phosphate. … Reactions 1-3 form the oxidative phase and generate NADPH and pentose 5-phosphate.
Drugs: Adenosine phosphate · ATP · Magnesium cation · Iron · 5-O-phosphono-alpha-D-ribofuranosyl diphosphate · D-glyceraldehyde 3-phosphate +4 moreEnzymes: Fructose-1,6-bisphosphatase 1, Fructose-bisphosphate aldolase A