| D007651 |
Keto Acids |
Carboxylic acids that contain a KETONE group. |
Oxo Acids,Oxoacids,Acids, Keto,Acids, Oxo |
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| D007655 |
Ketoglutarate Dehydrogenase Complex |
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2-Keto-4-Hydroxyglutarate Dehydrogenase,2-Oxoglutarate Dehydrogenase,2-Oxoglutarate Dehydrogenase Complex,Oxoglutarate Dehydrogenase,alpha-Ketoglutarate Dehydrogenase,alpha-Ketoglutarate Dehydrogenase Complex,2 Keto 4 Hydroxyglutarate Dehydrogenase,2 Oxoglutarate Dehydrogenase,2 Oxoglutarate Dehydrogenase Complex,Complex, 2-Oxoglutarate Dehydrogenase,Complex, Ketoglutarate Dehydrogenase,Complex, alpha-Ketoglutarate Dehydrogenase,Dehydrogenase Complex, 2-Oxoglutarate,Dehydrogenase Complex, Ketoglutarate,Dehydrogenase Complex, alpha-Ketoglutarate,Dehydrogenase, 2-Keto-4-Hydroxyglutarate,Dehydrogenase, 2-Oxoglutarate,Dehydrogenase, Oxoglutarate,Dehydrogenase, alpha-Ketoglutarate,alpha Ketoglutarate Dehydrogenase,alpha Ketoglutarate Dehydrogenase Complex |
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| D007930 |
Leucine |
An essential branched-chain amino acid important for hemoglobin formation. |
L-Leucine,Leucine, L-Isomer,L-Isomer Leucine,Leucine, L Isomer |
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| D008375 |
Maple Syrup Urine Disease |
An autosomal recessive inherited disorder with multiple forms of phenotypic expression, caused by a defect in the oxidative decarboxylation of branched-chain amino acids (AMINO ACIDS, BRANCHED-CHAIN). These metabolites accumulate in body fluids and render a "maple syrup" odor. The disease is divided into classic, intermediate, intermittent, and thiamine responsive subtypes. The classic form presents in the first week of life with ketoacidosis, hypoglycemia, emesis, neonatal seizures, and hypertonia. The intermediate and intermittent forms present in childhood or later with acute episodes of ataxia and vomiting. (From Adams et al., Principles of Neurology, 6th ed, p936) |
Branched-Chain Ketoaciduria,Thiamine Responsive Maple Syrup Urine Disease,BCKD Deficiency,Branched-Chain alpha-Keto Acid Dehydrogenase Deficiency,Classic Maple Syrup Urine Disease,Classical Maple Syrup Urine Disease,Intermediate Maple Syrup Urine Disease,Intermittent Maple Syrup Urine Disease,Keto Acid Decarboxylase Deficiency,MSUD (Maple Syrup Urine Disease),Maple Syrup Urine Disease, Classic,Maple Syrup Urine Disease, Classical,Maple Syrup Urine Disease, Intermediate,Maple Syrup Urine Disease, Intermittent,Maple Syrup Urine Disease, Thiamine Responsive,Maple Syrup Urine Disease, Thiamine-Responsive,Branched Chain Ketoaciduria,Branched Chain alpha Keto Acid Dehydrogenase Deficiency,Branched-Chain Ketoacidurias,Ketoaciduria, Branched-Chain,Ketoacidurias, Branched-Chain |
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| D008933 |
Mitochondrial Swelling |
An increase in MITOCHONDRIAL VOLUME due to an influx of fluid; it occurs in hypotonic solutions due to osmotic pressure and in isotonic solutions as a result of altered permeability of the membranes of respiring mitochondria. |
Giant Mitochondria,Megamitochondria,Mitochondrial Hypertrophy,Giant Mitochondrias,Hypertrophy, Mitochondrial,Megamitochondrias,Mitochondria, Giant,Mitochondrial Hypertrophies,Swelling, Mitochondrial |
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| D009249 |
NADP |
Nicotinamide adenine dinucleotide phosphate. A coenzyme composed of ribosylnicotinamide 5'-phosphate (NMN) coupled by pyrophosphate linkage to the 5'-phosphate adenosine 2',5'-bisphosphate. It serves as an electron carrier in a number of reactions, being alternately oxidized (NADP+) and reduced (NADPH). (Dorland, 27th ed) |
Coenzyme II,Nicotinamide-Adenine Dinucleotide Phosphate,Triphosphopyridine Nucleotide,NADPH,Dinucleotide Phosphate, Nicotinamide-Adenine,Nicotinamide Adenine Dinucleotide Phosphate,Nucleotide, Triphosphopyridine,Phosphate, Nicotinamide-Adenine Dinucleotide |
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| D010101 |
Oxygen Consumption |
The rate at which oxygen is used by a tissue; microliters of oxygen STPD used per milligram of tissue per hour; the rate at which oxygen enters the blood from alveolar gas, equal in the steady state to the consumption of oxygen by tissue metabolism throughout the body. (Stedman, 25th ed, p346) |
Consumption, Oxygen,Consumptions, Oxygen,Oxygen Consumptions |
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| D001921 |
Brain |
The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM. |
Encephalon |
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| D002491 |
Central Nervous System Agents |
A class of drugs producing both physiological and psychological effects through a variety of mechanisms. They can be divided into "specific" agents, e.g., affecting an identifiable molecular mechanism unique to target cells bearing receptors for that agent, and "nonspecific" agents, those producing effects on different target cells and acting by diverse molecular mechanisms. Those with nonspecific mechanisms are generally further classed according to whether they produce behavioral depression or stimulation. Those with specific mechanisms are classed by locus of action or specific therapeutic use. (From Gilman AG, et al., Goodman and Gilman's The Pharmacological Basis of Therapeutics, 8th ed, p252) |
Central Nervous System Drugs |
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| D004579 |
Electron Transport |
The process by which ELECTRONS are transported from a reduced substrate to molecular OXYGEN. (From Bennington, Saunders Dictionary and Encyclopedia of Laboratory Medicine and Technology, 1984, p270) |
Respiratory Chain,Chain, Respiratory,Chains, Respiratory,Respiratory Chains,Transport, Electron |
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