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Multiple Carboxylase Deficiency, Neonatal or Early Onset Form
smpdb.ca/view/SMP0000564DiseaseThe BTD gene encodes for biotinidase and the HLCS gene encodes for holocarboxylase synthetase. … Holocarboxylase synthetase deficiency also called Multiple Carboxylase Deficiency, Neonatal or Early Onset Form, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder of either mutations
Multiple Carboxylase Deficiency, Neonatal or Early Onset Form
smpdb.ca/view/SMP0125778DiseaseThe BTD gene encodes for biotinidase and the HLCS gene encodes for holocarboxylase synthetase. … Holocarboxylase synthetase deficiency also called Multiple Carboxylase Deficiency, Neonatal or Early Onset Form, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder of either mutations
Fatty 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. … When ATP is needed, ATP may be generated by increasing fatty acid metabolism. Fatty acid metabolism is essentially the reverse reaction of fatty acid synthesis.
Drugs: Adenosine phosphate · NADH · ATP · Levocarnitine · Magnesium cation · Flavin adenine dinucleotide +2 moreEnzymes: Long-chain-fatty-acid--CoA ligase 1Gadopentetic acid Metabolism
smpdb.ca/view/SMP0126898MetabolicIt is a complex of gadolinium with a chelating agent, diethylenetriamine penta-acetic acid (DTPA), that is given to enhance the image in cranial and spinal MRIs. … Gadopentetic acid is a gadolinium compound used as a contrast agent in MRIs.
Drugs: Gadopentetic acidCitric Acid Cycle
smpdb.ca/view/SMP0000057MetabolicMetabolically, the citric acid cycle allows the release of energy (ultimately in the form of ATP) from carbohydrates, fats, and proteins through the oxidation of acetyl-CoA. … The citrate generated via the citric acid cycle can serve as an intermediate for fatty acid synthesis; alpha-ketoglutarate can serve as a precursor for glutamate, proline, and arginine; oxaloacetate can
Enzymes: Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrialFatty Acid Biosynthesis
smpdb.ca/view/SMP0000456MetabolicThe biosynthesis of fatty acids primarily occurs in liver and lactating mammary glands. … The production of palmitic acid can be summarized as the successive addition of two carbons to an initial acetyl moiety primer. After 7 cycles palimitic acid is released.
Drugs: Biotin · Acetoacetic acid · Butenoic Acid · Malonic acid · (R)-3-hydroxytetradecanoic acid · Lauric acid +8 moreEnzymes: Fatty acid synthaseGadoteric acid Metabolism
smpdb.ca/view/SMP0126924MetabolicGadoteric acid is indicated for intravenous use with magnetic resonance imaging (MRI) in brain (intracranial), spine and associated tissues in adult and pediatric patients (2 years of age and older) to … Gadoteric acid is a diagnostic contrast agent used with magnetic resonance imaging to provide contrast enhancement for intracranial and spinal lesions with abnormal blood brain barrier or abnormal vascularity
Drugs: Gadoteric acidSelenoamino Acid Metabolism
smpdb.ca/view/SMP0000029Metabolic3-phosphate (lysophosphatidic acid). … All phospholipids contain sn-glycerol-3-phosphate esterified with fatty acids at the sn-1 and sn-2 positions.
Arachidonic Acid Metabolism
smpdb.ca/view/SMP0000075MetabolicThis pathway describes the production and subsequent metabolism of arachidonic acid, an omega-6 fatty acid. … In resting cells arachidonic acid is present in the phospholipids (especially phosphatidylethanolamine and phosphatidylcholine) of membranes of the body’s cells, and is particularly abundant in the brain