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58 Cards in this Set
- Front
- Back
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RLF of glycolysis?
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phosphofructokinase-1 (PFK-1)
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RLF of gluconeogenesis?
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fructose 1,6 bisphosphatase
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RLF of TCA cycle?
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isocitrate dehydrogenase
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RLF of gylcogen synthesis?
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gylcogen synthse
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RLF of gylcogenolysis?
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gylcogen phosphorlyase
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RLF of HMP shunt? is it oxidative or nonoxidative? reversible or irreversible? product?
What is the second major enzyme of the HMP shunt? ox or nonox? irreversible or reversible? product? |
G6PD
oxidative irreversible NADPH (transketolase, nonoxidative, reversible, Ribose-5-phosphate) |
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RLF of de novo pyrmidine synthesis?
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aspartate transcarbamoylase
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RLF of de novo purine synthesis?
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glutamine-PRPP amidotransferase
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RLF of urea cycle?
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carbamoyl phosphate synthase 1
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RLF of fatty acid synthesis?
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acetyl-CoA carboxylase
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RLF of fatty acid oxidation?
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carnitine acyltransferase 1
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RLF of ketogenesis?
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HMG-CoA synthase
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RLF of cholesterol?
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HMG-CoA reductase
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What is the pathway, location and nitrogen source of CPS1?
What is the pathway, location and nitrogen source of CPS2? |
CPS1- urea cycle, mitochondria, ammonia
CPS2- pyrmidine synthesis, cytosol, glutamine |
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which enzyme charges tRNA?
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aminoacyl-tRNA synthetase
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which enzyme catalyzes the peptide bond formation?
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peptidyl transferase
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what happens in vit A deficiency?
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night blindness/dry skin
also more susceptible to measles? (Vit A has been shown to help treat measles) |
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what happens in Vit A excess?
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retinoic acid- teratogen- cleft palate, cardiac
arthralgias, fatigue, headaches, skin changes, sore throat, alopecia |
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Vitamin B1- what is the active form? what 4 reactions is it a coenzyme for?
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thamine pyrophosphate
1. pyruvate dehydrogenase 2. a-ketogulterate dehydrogenase (TCA_ 3. thansketolase (HMP) 4. branched chain AA dehydrogenase |
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three situations seen in vit b1 deficiency:
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1. wernicke-korsakoff syndrome
(confusion, ataxia, ophthalmoplegia/confabulation, personality change, memory loss) 2. wet beriberi- high output heart failure 3. dry beriberi- polyneuritis, muscle wasting |
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what part of the brain does wernicke-korsakoff syndrome effect?
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mamillary bodies
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what is the name of vit b2?
reaction is vit b2 a cofactor in? |
riboflavin
cofactor in oxidation and reduction makes FAD and FMN |
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what are the two major symptoms seen in B2 deficiency?
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cheilosis- fissuring, inflammation and scaling of lips
corneal vascularization |
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what is the precursor of niacin? what is niacin a constituent in?
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tryptophan
NAD+ and NADP+ |
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what vitamin does synthesis of niacin require?
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vit b6
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what does deficiency in B3 result in? (2)
what conditions can cause b3 deficiency? (3) |
glossitis
pallegera- diarrhea, dermatitis, dementia 1. Hartnups disease (decreased tryptophan absorption) 2. malignant carcinoid syndrome (increase try metabolism) 3. INH- decreases B6 |
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signs of excess niacin?
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facial flushing
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vit B5 is an essential component of what?
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pantothenate is an essential component of CoA and FA synthase
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vit B6 functions in which reactions? 5 reactions
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1. transamination (AST and ALT)
2. decarboxylation reactions 3. gylcogen phosphorylase 4. heme synthesis 5. formation of B3 |
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signs of b6 deficiency?
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convulsions, hyperirritability, peripheral neuropathy (INH and OCPs)
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B12 is a cofactor in which 2 reactions?
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1. homocysteine methlytransfrase
2. methylmalonyl-CoA mutase |
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deficiency of b12? (2)
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macrocytic, megalobalstic anemia
neurologic symptoms (paresthesis) can be irreversibe |
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signs of folate deficiency?
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most common deficiency in the US.
macrocytic, megaloblastic anemia |
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what is the function of SAM, what reaction is it used in and what vitamin is it dependent on?
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1. SAM is a methyl transferring unit
2. is it used in the regeneration of methionine 3. dependent on B12 |
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Biotin is a cofactor in which reactions? 3
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1. pyruvate carboxylase (pyruvate-->oxalacetate)
2. acetyl co-a carboxylase (acetlyCoA-->malonyl-CoA) 3. propionyl CoA carboxylase (propionyl CoA-->methylmalonyl CoA) |
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signs of deficiency of Vit C?
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scurvy- swollen gums, bruising, anemia, poor wound healing
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vitamin K is important in which clotting factors?
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Protein C S
10 9 7 2 |
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enzyme: pyruvate-->acetly-CoA
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pyruvate dehudrogenase
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enzyme: pyruvate-->oxaloacetate
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pyruvate carboxylase
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enzyme: pyruvate--> alanine
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ALT
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enzyme pyruvate-->lactate
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lactate dehydrogenase
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what cofactors are required in the pyruvate dehydorgeanse complex? 5
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1. TPP
2. lipoic acid 3. CoA 4. FAD 5. NAD |
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compare the locations of hexokinase and glucokinase...
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hexokinase: all tissues- no insulin dependent
glucokinase: liver and b cells of pancreas- insulin dependent |
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is it worse to have a fructokinase or aldolase B deficiency?
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aldolase B- causes hereditary fructose intolerance- baby being switched to fruit juices
fructokinase- asx, fructose in urine |
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what two errors happen in galactose metabolism deficiencies?
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1. classic galactosemia- absence of glalctose-1-phosphate uridyltransferase - toxic substances accumulate, MR, jaundice, infant cataracts
2. glalctokinase deficiency- deficiency in glalctokinase C, failure to track objects or have a social smile. |
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what are the glucogenic essential AAs?
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met, val, arg, his
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what are the gulcogenic/ketogenic essential AAs?
4 |
ile, phe, thr, trp
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what are the ketogenic essential AAs?
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lue lys
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what enzmye is absent in PKU?
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phenalanine hydroxylase
or tetrahydrobiopterin cofactor |
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what enzyme is absent in alkaptonuria?
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homogentisic acid oxidase
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enzyme: tyrosine-->fumarate
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homogentisic oxidase
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what is absent in albinism?
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tyrosinase
or defective tyrosine transporters |
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what is the most common urea cycle disorder? what are the findings in the blood?
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ornithine transcarbamoylase deficiency
find: orotic acid in the blood |
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what enzyme is deficient in orotic acid deficiency? 2
what are the clinical manifestations? |
orotic acid phosphoribosyltransferase or orotidine 5-phosphate
megalobalstic anemia (does not improve with B12 addition), failure to thrive |
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enzyme in von gierkes disease?
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glucose-6-phosphatase
type I, lactic acid bulid-up |
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enzyme deficiency in pompe's disease?
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lysosomal a1,4 glucosidase
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enzyme deficieny in cori's disease??
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debranching enzyme
(a1,6 glucosidase) like type I, no lactate deficiency |
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deficiency in Mc Ardles disease?
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skeletal muscle glycogen phosphorylase
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