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Definition
| (pesticide) Inhibit Electron Transport. Inhibit NADH dehydrogenase in complex 1 |
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Term
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Definition
| Bacterial anti biotic )Inhibit Electron Transport. Inhibit NADH dehydrogenase in complex 1 (Rotenone and amytal(barbituate) |
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Definition
| Barbituate – Inhibit Electron Transport. Inhibit NADH dehydrogenase in complex 1 ( Like Rotenone and piercidin A) |
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Term
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Definition
| Antibiotic-inhibits cytochrome B of cytochrome reductast(complex 3) |
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Term
| Inhibits Cytochrome Oxidase |
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Definition
| (Complex 4 inhibitor) Co, azide, hydrogen sulfide, cyanide |
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Term
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Definition
| Streptomyces antibiotic: inhibits ATP synthase (Complex V) |
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Definition
| Similar to oligomycin, inhibits ADP/ATP transport |
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Definition
| Similar to oligomycin, inhibits ADP/ATP transport |
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Term
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Definition
| AKA Uncoupling protien UCP. Regulated uncoupler of oxidative phosporylation. (Deacrease ATP synthesis via creating “proton Leak” allowing H+ to re-enter the mitochondrial matrix without capturing energy. ) Used to Generate heat. Physiological function of brown adipose tissue. Aound neck and upper back in babies. Biological heat bap |
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Definition
| Uncoupler of Oxidative Phos.(Deacrease ATP synthesis via creating “proton Leak” allowing H+ to re-enter the mitochondrial matrix without capturing energy. ) |
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Term
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Definition
| Uncoupler of oxidative phosporylation (Deacrease ATP synthesis via creating “proton Leak” allowing H+ to re-enter the mitochondrial matrix without capturing energy. ) |
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Term
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Definition
| CHANNEL FORMING IONOPHORE . Uncoupler. Channel forming ioniphore. Makes a pore through mitochondrial inner membrane. Allows for dissipation of H+ gradient |
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Term
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Definition
| MOBILE CARRIER IONOPHORE K+ Uncoupler. Antibiotic from streptomyces strains. Move back and forth through the membrane. Typicalled associated with carrying K+ across bilayer. |
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Definition
| Type 1 GSD: Enzyme: (1a) Glucose 6-phosphatase in ER Membrane (1b) Deficiency in glucose 6 phosphate translocase. BOTH glycogen degredation and gluconeogenesis impaired(Both end in glucose 6 phosphate-->Need to be converted) SEVERE HYPOGLYCEMIA DURING FASTING. Hepatomegaly, Lactic adidemia(buildup of lactate because not being used for gluconeogenisis.)Untreated: Progressive live and kidney disease TREATMENT: regularadministration of uncooked corn starch or nocturnal gastric glucose infusions. |
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Definition
| Type 2 GSD: Deficiency in lysosomal acid a-1-4 glucosidase. Lysosomal glycogen accumilation in the heart muscle and liver. (muscular and lysosomal storage disease) WEAKNESS, progressive neuromuscular disorder, variation of symptoms. Normal blood glucose levels. INFANT MOST SERVER: CARDIO MEGALY, myopathy, Hepatomegaly.--> Earyl death in first year. TREATMENT: Enzyme replacement infusion. ?able |
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Term
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Definition
| Type 3 GSD: Debranching enzyme deficiency:(Forbes/limit dextrinosis)- Mild Hypoglycemia and hepatomegaly. Short outer branches of glycogen. Muscle weaknes, hypotonia and cardiomyopathy. Wheelchair bound by 50-60 years old. Muscular dystrophy |
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Term
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Definition
| Typer 4 GSD:Branching enzyme definciency. Abnormal glycogen-> scarring by the attack of the body's immune system, INFANTILE CIRROSIS, death by age 6 |
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Definition
| (Type V GSD): Def. Myophosphorylase. Cant break down musc. Glycogen. Temporary weakness/cramping after exercise. Lethargic child. Rhabdo after forced exercise(no ATP) High Serum MM, NO RAISE LACTATE after strenous exercise. Myoglobiniema/Myoglobinuria |
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Definition
| VI GSD: Def of Hepatophosphorylase: Hepatomegaly and growth retardation. Mild hypoglycemia and hyperketonemia. Less sever than Von Gierk cause gluconeogenesis still running. |
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Term
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Definition
| Very Rare: Defin in PFK 1(M type) in muscle and RBC (Muscle has L type so 50 % can preform glycolysis. Muscle cramping due to lack of ATP. No raise in Lactate. Hemolysis b/c 50 % PFK1 deficiency in RBC. |
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Term
| Pyruvate Dehydrogenase deficiency |
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Definition
| Increased pyruvate==>Lactic acide and alanine. Decreased Acetyl CoA and severe redecuction in ATP. Clincally: Lactic acidosis, neurological defects, myopathy, usually fatal at early age. |
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Term
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Definition
| Pyruvate Dehydrogenase Deficiency: Binds to lopic acid which is needed by dyhydrolipoyl transacetylase. Results in lactic acid, neurological defects, brain particuallary, can lead to death.(can also effect Ketoglutarate DH. |
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Term
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Definition
| Pesticide. Inhibits Aconitase. Malfunction in TCA |
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Term
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Definition
| Inhibits Succinate Dehydrogenase. Malfunction in TCA |
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Term
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Definition
| Defieciencies in B vitamins (niacin,riboflavin,thiamine) inhibit TCA |
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Term
| Lebers Hereditary Optic Neuropathy |
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Definition
| Defect in NADH Dehydrogenase(ETC) |
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Term
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Definition
| (M.M.E.L.A.S. Mitochodrial myopathy Hereditary Mitochondrial Disease: Mitochondrial Miopathy, Encephalopathy. Lactic acidosis, Stroke Like Episods. May present only as diabetes mellitus |
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Term
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Definition
| Hereditary Mitochondrial disease. M.E.R.F.F. Myoclonic epilepsy, ragged red fibers, ataxia, sensorinueral deafness |
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Term
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Definition
| Hereditary mitochondrial disease. Early onset eye problems. Retinal pigmentation. Bad motor movements in eyes Early onset with heart block, retinal |
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Term
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Definition
| Amyotrophic Lateral Schlerosis/ Lou Gehrigs- Deficiency of superoxide dismutase. Lack of defense against superoxide. Degenerative disease for both upper and lower motor nuerons. Muscle weakness, atrophy, dysphagia, dysarthria(motor Speech disorder), hyperreflexia. Leads to DEATH |
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Definition
| Forms Oh- radicals. H202 + 02- +H+ = Oh-, h20, 02....Hydrogen peroxied + superoxide = hydroxyl radical, water and oxygen |
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Term
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Definition
| OH- Radicals from Hydrogen peroxide (H202) plus Ferous ion Fe+2 (ferrous for fenton) |
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Term
| Chronic Granulomas Disease |
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Definition
| (Aka Bridges-good syndrome) Deficient NADPH oxidase. Lack of superoxide formation in leukocytes. Severe reccurent infections. (Fungi or bacterial) |
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Term
| Myeloperoxidase (MPO) Deficiency |
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Definition
| Genetic Disorder. Myelo-peroxidase deficiency. Cannot convert H202 to Hypoclorous acid in phagolysosomes. Presents with reccurent infections. (Fungal-candida albicans) Oral, genital, and systemic funga infections. |
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Term
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Definition
| Malabsorption disease. Systemic Infectious disease. From Bacterium(troph whipple) |
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Term
| (histo)Traveler's diarrhea |
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Definition
| Malabsorption syndrome. Bacterial infection |
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Term
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Definition
| Malabsorbtion disease. Abnormal flatteing of villi and inflamaiton of small intestine lining |
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Term
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Definition
| Lysosomal storage disease. Genetic Defect in lysosomal hydrolase.Cant break down gangliodes |
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Term
| Zellweger Syndrome (Histo) |
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Definition
| Peroxisome deficiency: Causes abnormalities in mylenation of nerve cells. (B/c Perozisomes(Micro bodies) are important in fat metabolism. |
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Term
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Definition
| Inhibit Phosphodiesterase. Therefore keeping cAMP active after Adenlycyclase activation of it. |
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Term
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Definition
| Ribosylates ADP Gs ..Inactivates GTPase therefor leaving it active and cAMP and adenylate Cylases Active.Protein Kinase A. Can lead to exended opening of CFTR channel because of hyper phosphorylation |
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Term
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Definition
| Ribosylates Gi : Keeps inhibition active. |
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Term
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Definition
Thiamine deficiency (B1)leads to interuption of Pyruvate Dehydrogenase. Can manifest with Ataxia, Opthalmolplasia(rapid eye movements) memory loss, cerebral hemorage. Usually due to Alholics(blocks thiamine absorption) or malnurished peopel. Eventually lack of ATP leads to server cardiac problems and increased cardiac output-> Wet Beri Beri
A-ketoglutamarate also needs vit B |
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