Term
|
Definition
| uses ATP to add high-energy phosphate |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| transfers CO2 with biotin |
|
|
Term
| which cells lack sorbitol dehydrogenase |
|
Definition
| schwann, lens, retina, and kidneys |
|
|
Term
| cells have both aldose reductase and sorbitol dehydrogenase |
|
Definition
| liver, ovaries and seminal vesicles |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| RLE for glycogen synthesis |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| RLE for pyrimidine synthesis |
|
Definition
|
|
Term
|
Definition
| glutamine-PRPP amidotransferase |
|
|
Term
|
Definition
| carbamoyl phosphate synthetase I |
|
|
Term
| RLE for fatty acid synthesis |
|
Definition
|
|
Term
| RLE for fatty acid oxidation |
|
Definition
| carnitine acyltransferase I |
|
|
Term
|
Definition
|
|
Term
| RLE for cholesterol synthesis |
|
Definition
|
|
Term
| glucose -> glucose-6-phosphate |
|
Definition
|
|
Term
| fructose-6-phosphate -> fructose-1,6-BP |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| glucose-6-phosphate -> glucose |
|
Definition
|
|
Term
| fructose-1,6-BP -> fructoose-6-phosphate |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| most common glycolysis deficiency enzyme |
|
Definition
|
|
Term
| glyclosis deficiency presentation |
|
Definition
|
|
Term
| malate shuttle yields ___ ATP |
|
Definition
|
|
Term
| glycerol-3-phosphate yields ___ ATP |
|
Definition
|
|
Term
| anaerobi glycolysis yields ___ ATP |
|
Definition
|
|
Term
| Hexo vs glucokinase: low Km |
|
Definition
|
|
Term
| Hexo vs glucokinase: high Km |
|
Definition
|
|
Term
| Hexo vs glucokinase: low Vmax |
|
Definition
|
|
Term
| Hexo vs glucokinase: high Vmax |
|
Definition
|
|
Term
| Hexo vs glucokinase: induced by insulin |
|
Definition
|
|
Term
| Hexo vs glucokinase: in all cells |
|
Definition
|
|
Term
| Hexo vs glucokinase: liver and beta cells |
|
Definition
|
|
Term
|
Definition
|
|
Term
| inhibition of glucokinase |
|
Definition
|
|
Term
| activation of phosphofructokinase |
|
Definition
|
|
Term
| inhibition of phosphofructokinase |
|
Definition
|
|
Term
| inhibition of pyruvate kinase |
|
Definition
|
|
Term
| activation of pyruvate kinase |
|
Definition
|
|
Term
| pyruvate dehydrogenase inhibtion |
|
Definition
|
|
Term
| increased glucagon effect on glycolysis |
|
Definition
|
|
Term
| increased insulin effect on glycolysis |
|
Definition
| more glycolysis (more PFK2) |
|
|
Term
| cofactors for pyruvate dehydrogenase |
|
Definition
| thiamine, Lipoic acid, CoA, FAD, NAD |
|
|
Term
| vomiting, rice water stools, garlic breath |
|
Definition
|
|
Term
|
Definition
|
|
Term
| pyruvate dehydrogenase activated by |
|
Definition
|
|
Term
|
Definition
| mammary glands, liver, adrenal cortex and RBCs |
|
|
Term
| glucose-6-p -> ribulose-5-P |
|
Definition
|
|
Term
| ribulose-5-P is used for ____ |
|
Definition
|
|
Term
| ribulose-5-P -> ribose-6-P |
|
Definition
|
|
Term
| oxidative burst enzymes in neutrophils |
|
Definition
| O2 --NADPH oxidase--> O2- --Superoxide dismuates--> H2O2 --myeloperoxidase--> HOCl- |
|
|
Term
| CGD increased infection with |
|
Definition
|
|
Term
| heinz bodies and bite cells |
|
Definition
|
|
Term
|
Definition
| poor RBC defense against ROS -> hemolytic anemia |
|
|
Term
| ppts of hemolysis in G6PD deficiency |
|
Definition
| fava beans, sulfonamides, primaquine, anti-TB |
|
|
Term
|
Definition
|
|
Term
| fructose in blood and urine |
|
Definition
|
|
Term
|
Definition
|
|
Term
| hypoglycemia, jaundice, cirrhosis, vomiting |
|
Definition
|
|
Term
|
Definition
| decrease intake of both fructose and sucrose |
|
|
Term
| deficiency of galactokinase |
|
Definition
|
|
Term
| galactose in blood and urine, infantile cataracts |
|
Definition
|
|
Term
| deficiency of galactose-1-phosphate uridyltransferase |
|
Definition
|
|
Term
| falure to thrive, jaundice, hepatomegaly, infantile cataracts, MR |
|
Definition
|
|
Term
|
Definition
| exclude galactose and lactose |
|
|
Term
| bloating, cramps and osmotic diarrhea |
|
Definition
|
|
Term
| symptoms of pyruvate dehydrogenase deficiency |
|
Definition
| lactic acidosis because backup of pyruvate and alanine |
|
|
Term
| tx of pyruvate dehydrogenase deficiency |
|
Definition
| increase intake of ketogenic nutrients (high fat or increased lysine and leucine) |
|
|
Term
|
Definition
| alanine, lactate, OAA acetyl-CoA |
|
|
Term
| three regulated enzymes of TCA |
|
Definition
| citrate synthase, isocitrate dehydrogenase, alpha-KG dehydrogenase |
|
|
Term
| citrate synthase inhibition |
|
Definition
|
|
Term
isocitrate dehydrognase
- inhibition
- activation |
|
Definition
|
|
Term
| alpha-KG dehydrogenase inhibition |
|
Definition
| succinyl-CoA, NADH, and ATP |
|
|
Term
| inhibition of complex I in electron transport chain |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| effets of electron transport inhibitors |
|
Definition
| directly inhibit -> decrease proton gradient and block of ATP synthesis |
|
|
Term
|
Definition
| ologmycin, increase proton gradient and no ATP produced because electron transport stops |
|
|
Term
|
Definition
| 2,4-DNP, ASA, thermogenin |
|
|
Term
| effects of uncoupling agents |
|
Definition
| increase permeability of membrane -> decrease proton gradient and increase O2 consumption |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| fructose-1,6-BP -> fructose-6P |
|
Definition
| fructose-1,6-bisophosphatase |
|
|
Term
|
Definition
|
|
Term
| which cells cannot participate in gluconeogenesis |
|
Definition
|
|
Term
| odd and even FA difference |
|
Definition
odd = can enter TCA, gluconeogenesis and as glucose source
even = only acetyl-CoA equivalents |
|
|
Term
|
Definition
mm= glucose -> lactate
lactate in blood to liver
liver = lactate -> glucose |
|
|
Term
|
Definition
mm= glucose -> pyr -> ala
liver = ala -> pyruvate -> glucose |
|
|
Term
| two major carriers of Nitrogen |
|
Definition
|
|
Term
|
Definition
|
|
Term
| glucose-2-phosphatase deficiency |
|
Definition
|
|
Term
| severe fasting hypoglycemia |
|
Definition
|
|
Term
|
Definition
|
|
Term
| increase glycogen in liver, hepatomegaly |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| lysosomal alpha-1,4-glucosidase deficiency |
|
Definition
|
|
Term
| milk don von gierke with normal lactate |
|
Definition
|
|
Term
| alpha-1,6-glucosidase deficiency |
|
Definition
|
|
Term
|
Definition
|
|
Term
| mm cramps, myoglobinuria with strenuous exercise |
|
Definition
|
|
Term
| skeeltaml mm glycogen phosphorylase deficiency |
|
Definition
|
|
Term
|
Definition
| acetoacetate and beta-hydroxybutyrate |
|
|
Term
| ketone used in mm and brain |
|
Definition
|
|
Term
|
Definition
|
|
Term
| how are ketones metabolized by the brain |
|
Definition
|
|
Term
| what metabolic scenario favors the synthesis of ketone bodies? |
|
Definition
| acetyl-CoA exceeds O2 capacity of the TCA |
|
|
Term
|
Definition
| glycolysis and aerobic respiration |
|
|
Term
| metabolism in fasting state between meels |
|
Definition
| glycogenolysis, gluconeogenesis, adipose release of FA |
|
|
Term
| starvation 1-3 days metabolism |
|
Definition
| glycogenolysis, FFA, gluconeogenesis |
|
|
Term
| starvation after day 3 metabolism |
|
Definition
| ketone bodies become main source of energy for brain and heart |
|
|
Term
|
Definition
| ornithine transcarbamoylase |
|
|
Term
|
Definition
|
|
Term
|
Definition
| PVT TIM HALL = phe, Val, Trp, The, Ile, Met, His, Arg, Leu, Lys |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| liver disease, urea cycle deficiency |
|
|
Term
| tremor, vomitin, slurring of speech, cerebral edema, blurring of vision |
|
Definition
|
|
Term
|
Definition
| benzoate or phenylbutyrate, lactuloase = hepatic encephalopathy |
|
|
Term
| most common urea cycle disorder |
|
Definition
| ornithin transcarbamoylase deficiency |
|
|
Term
| orotic acid in blood and urine, decreased BUN, increased NH4 |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| location of beta-oxidation |
|
Definition
|
|
Term
| weakness, hypoketotic hypoglycemia |
|
Definition
|
|
Term
| increased dicarboxylic acids, increased glucose and ketones |
|
Definition
| acyl-coa dehydrogenase deficiency |
|
|
Term
|
Definition
|
|
Term
| what inhibits HMG-CoA reductase? |
|
Definition
|
|
Term
|
Definition
| chylomicron, remnant, VLDL, IDL, HDL |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| lipoprotein lipase cofactor |
|
Definition
|
|
Term
| mediates chylomicron secretion |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| delivers dietary TGs to peripheral tissue |
|
Definition
|
|
Term
| secreted by intestinaal epithelial cels |
|
Definition
|
|
Term
| delivers hepatic TGs to peripheral tissue |
|
Definition
|
|
Term
|
Definition
|
|
Term
| delivers TG and cholesterol to liver |
|
Definition
|
|
Term
|
Definition
|
|
Term
| delivers hepatic cholesterol to peripheral tissue |
|
Definition
|
|
Term
|
Definition
|
|
Term
| reverse cholesterol transport from periphery to liver |
|
Definition
|
|
Term
| acts as a repository for apoC and apoE |
|
Definition
|
|
Term
| secreted from liver and intestine |
|
Definition
|
|
Term
|
Definition
| type I = hyperchylomicronemia |
|
|
Term
| deficiency in hyperchylomicronemia |
|
Definition
| lipoprotein lipase deficiency or altered apolipoprotein CII |
|
|
Term
| pancreatitis, hepatosmplenomegaly and eruptive xanthomas |
|
Definition
| type I = hyperchylomicronemia |
|
|
Term
|
Definition
| type II = familial hypercholesterolemia |
|
|
Term
| deficiency in familial hypercholesterolemia |
|
Definition
|
|
Term
| accelerated atherosclerosis, tendon anthems an corneal ulcers |
|
Definition
| type II = familial hypercholesterolemia |
|
|
Term
|
Definition
| type IV = hypertriglyceridemia |
|
|
Term
| hepatic overproduction of VLDL leading to pancreatitis |
|
Definition
| type IV = hypertriglyceridemia |
|
|
Term
| accumulation of fats in enterocyte because cannot export as chylomicrons |
|
Definition
|
|
Term
| failure to thieve, acanthocytosis, steatorrhea, ataxia and night blindness |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| phenylkenonuria deficiency |
|
Definition
| phenylalanine hydroxylase or decreased tetrahydrobiopterin |
|
|
Term
| mental retardation, eczema, seizures, must body odor, fair skin |
|
Definition
|
|
Term
| microcephaly, growth retardation, congenital heart defects |
|
Definition
|
|
Term
| deficiency in alkaptonuria |
|
Definition
|
|
Term
| dark CT, browht sclera, urine turns black |
|
Definition
|
|
Term
|
Definition
| tyrosinase or defectiv eytrosine transporters |
|
|
Term
| mental retardation, tal stature, lens sublux, atherosclerosis |
|
Definition
|
|
Term
| homocystinuria deficiencies |
|
Definition
1- cystathionine synthase
2- dec affinity of cystathionine synthase for pyridoxal phosphate
3- homocysteine methyltransferase deficiency |
|
|
Term
|
Definition
|
|
Term
| blocked degradation of branched AA (Ile, Leu, Val) |
|
Definition
| maple syrup urine disease |
|
|
Term
| sweet urine, mental retardation, death |
|
Definition
| maple syrup urine disease |
|
|
Term
|
Definition
| defective AA transporter on renal and intestinal cells -> pellagra |
|
|
