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52 Cards in this Set
- Front
- Back
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what is the rate limiting reaction in cholesterol synthesis?
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HMG Co reductase
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name this condition
mousy odor, tyrosine missing, hence it must be supplied in the diet, can diagnose by amniocentesis and finding the abnormal gene; eliminate phenylalanine from diet |
PKU
**no nutrasweet!!! |
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what is I cell disease?
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inability to phsophorylate the mannose residues of potential lysosomal enzymes located in Golgi apparatus, hence they cannot be taken up by the lysosomes to degrade complex substrates
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explain the number of glucoses necessary to build palmitic acid a 16 carbon compound
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4 glucoses, each glucose produces 2 acetyl CoA the latter containing 2 carbons each
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what is the result of a lack of insulin in DKA?
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decreased glycolysis, glycogenesis, fatty acid synthesis, storage of fat in adipose
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name some uncoupling agents and explain what they do
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alcohol and salicylates
produces brown fat from increased heat from reactions trying to increase the generation of more protons to make ATP |
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what is Von Gierke's disease?
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decreased glucose 6-phosphatase (gluconeogenic enzyme) with decrease in glucsose (fasting hypoglycemia) and increase in glucose 6-phosphate with production of normal glycogen in the liver and kidneys; stimulation tests with glucagon, fructose, etc. cannot increase the glucose levels owing to the missing enzyme
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explain the biochemical processes in both cytosol and mitochondria
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urea cycle, heme synthesis, gluconeogenesis
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what dietary alteration would you suggest for a female that has pheochromocytoma?
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decreased phenylalanine (essential AA) and tyrosine (not an essential AA) in the diet
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how would you counsel a patient with PKU who was pregnant?
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low in phenylalanine and high in tyrosine-->avoid nutrasweet since it contains aspartate and phenylalanine
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what is Lesch Nyhan?
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SXR with absent HGPRT, self mutilation, hyperuricemia, mental retardation
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where is glucokinase located?
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only in the liver
High Vm and high Km, not inhibited by glucose 6-phosphate hexokinase, in all tissues inhibited by glucose 6-phosphate; low Vm and low Km |
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what tissue can metabolize branched chain amino acids in maple syrup urine disease?
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only muscle
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know glycogenolysis!
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.....
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what is the key enzyme in gluconeogenesis?
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fructose 1, 6 bisphosphatase
catalyzes the conversion of fructose 1,6-bisphosphate to fructose 6-phosphatase |
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what the locations of glucose 6-phosphatase?
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liver, kidney, intestinal epithelium (lesser extent than others) absent in von Gierke's disease!
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what does the carnitine shuttle carry?
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even chained fatty acids
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what does the malate shuttle carry?
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NADH
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what are the functions of LDL?
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vitamin D synthesis, other steroid, cell membranes, synthesis of bile salts/acids
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Uses of acetyl CoA?
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FA syntheisis
CH synthesis ketone body synthesis ***not a substrate for gluconeogenesis |
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discuss Km and Vmax Lineweaver Burke
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competitive vs. non-competitive inhibitors, competitive (i.e. alcohol dehydrogenase binding ethanol, methanol, ethylene glycol at the same binding site) has no change in Vm but an increase in Km (lower affinity for ethanol, increasing ethanol reverses the inhibtion)
non-competitive inhibitor (organophosphates and effect on acetylcholinesteratse does not bind to the same site as acetylcholine, binds to another site on the enzyme that decreases velocity of the reaction) has a decrease in Vm, but the Km remains the same, since the substrate still binds to the normal site |
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what is the relationship between fatty acid length and energy production?
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....
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what is the method of eliminating ammonia?
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urea cycle
**located in the hepatocyte |
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what is the differential if epinephrine is given and only small branched chains found?
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debrancher deficiency
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draw cholesterol synthesis
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-----
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what is the rate limiting step in glycogenolysis?
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glycogen phosphorylase
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what is the origin of apolipoprotein 100?
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liver
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what is the origin of apolipoprotein 48?
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intestine
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what is the reason why liver can not use ketones for fuel?
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liver cannot activate acetoacetate in the mitochondria which requires succinyl CoA; acetoacetate CoA transferase (a thiotransferase enzyme) in order to convert AcAc into acetoacetyl CoA
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what is wrong in McArdles disease?
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absent muscle phosphorylase, increased glycogen in muscle, no increase in lactic acid after exercise
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A pregnant woman is a beer drinker---> what supplements does she need?
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folate because alcohol increases loss of folate in urine and stool which offsets the amount of folate present in beer; furthermore she would probably be taken off beer because of fetal alcohol syndrome and would need folate; iron is not affected by beer drinking
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what is the key hormone of the fed state?
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insulin
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what is the key hormone of the fasting state?
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glucagon
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know all the lysosomal storage diseases
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....
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mannose 6-phosphate is involved in what?
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mannose 6-phosphate is involved in transfer of dolichol (lipid) in the RER in the synthesis of O-linked glycosides
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what are the reactions involving NAD/NADH and NADP/NADPH?
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...
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what is the major source of NADPH?
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HMP shunt; malate dehydrogenase reaction to a lesser extent; NADPH supplies reducing equivalents
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Mutation changes an amino acid sequence--> which one would have the greatest effect on migration in a serum protein electrophoriesis?
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one with the most negative charges (most acidic) glutamine; one that would remain closest to the anode (negative pole) is the most basic amino acid--arginine
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what is the mechanism of ketoacidosis in DKA
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increased beta-oxidation of fatty acids and production of acetyl CoA which is used by the liver to synthesize ketone bodies
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where is the promotor location?
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linear gene drawing with labels; pick upstream location
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what is the energy source for protein synthesis?
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GTP
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what isoenzymes have 2 genes and 4 subunits?
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LDH
5 isotypes = LLLL, LLLH, LLHH, LHHH, HHHH |
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name the second messanger
atrial natriuretic peptide |
cGMP
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name the second messanger
insulin |
tyrosine kinase
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name the second messanger
nicotinic |
ion channels
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what is the best method of detecting relatedness of a new bacteria?
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restriction fragment lenght polymophism
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what is the enzyme used for PCR?
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DNA polymerase
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what is the source of glucose in a brain in starvation?
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alanine--> transaminated into pyruvate
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what is the purpose of glutamine?
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carriers ammonia in nontoxic form
**most abundant amino acid |
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what is the purpose of tyrosine?
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synthesis of melanin, synthesis of neurotransmitters (dopamine, Nor, Epi) decreased in PKU (must supply tyrosine in diet)
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what is they purpose of glycine?
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inhibitory neurotransmitter blocked by tetanus toxin, synthesis of delta aminolevulinic acid in heme synthesis, synthesis of bile acid/salts
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what is the biochemistry of hepatic encephalopathy?
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increased in aromatic amino acids--> phenylalanine, tyrosine, tryptophan (menonic PTT) leads to increased synthesis of false neurotransmitters (GABA, ocopamine) in hepatic encephalopathy; branched chain amino acids inhibit synthesis of false neurotransmitters---reason why they are given for treatment
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