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12 Cards in this Set

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What are the 10 essential amino acids?
"Any Help In Learning these Little Molecules Proves Truly Valuable."

Arginine (urea cycle)
Histidine
Isoleucine
Leucine (ketogenic)
Threonine
Lycine (ketogenic)
Methionine
Phenylalanine
Tryptophan
Valine
What are the substances used in the urea cycle?
" ordinarily, careless crappers are also frivolous about urination"

ornithine
carbamoyl phosphate
citrulline
aspartate
argininosuccinate
fumarate
arginine
urea
What are the substances used in the TCA cycle?
" cindy is kinky so she fornicates more often"

citrate
isocitrate
ketoglutarate (alpha-)
succinyl coa
succinate
fumarate
malate
oxaloacetate
What are the familial dyslipidemias?
1=chylo (lipop.lipase/apoCII)
2=LDL (a=receptor, b=production)
3=IDL (apoE)
4=VLDL (prod)
5=VLDL and chylo (prod/clear)
What crystals are found in gout?
monosodium urate (not uric acid!)
What is different between endo and exopeptidases?
endo: cleave aminobond within protein

exo: cleav ends of -> aminopeptidase or carboxypeptidase

nb amino acid sequences are given in N-C order
Where are glut1-5 found?
1= RBC, brain vessels, kidney, colon
2= liver, pancreas
3= neurons, placenta, testes
4= muscle (only one influenced by insulin)
5= sm intestine, testes, sperm (=fructose transporter)
What are serine proteases?
cleave proteins and have serine residue

trypsin, chymotrypsin, thrombin, plasminogen, plasmin, elastase, cathepsin G
What does albumin consist of en what is essential in the making?
glycoprotein

protein, carbohydrate chains synthesized on dolichol
What is Hartnups disease
epithelial transport neutral AA -> sx vlg pellagra
What are basics of purines and pyrimidines
purine: aspartate, co2, glycine, n10-formyl-tetrahydrofolate, glutamine

pyrimidine: carbamoyl phosphate, aspartate (source of cleaved co2)
Which type of disease in newborns presents with:
a) severe hyperammonemia and respiratory alkalosis
b) hypoketotic hypoglycemia, hyperammonemia and cardiomyopathy
c) hypoglycemia, lactic/ketoacidosis and hepatosplenomegaly
d) feeding difficulties, lethargy, seizures, coma, renal/liver disease
e) metabolic acidosis with ketosis, elevated lactate, mild hyperammonemia, vomiting, encephalopathy, neutropenia, thrombocytopenia
a) urea cycle defects
b) # fatty acid oxidation
c) # carbohydrate metabolism
d) aminoacidopathy
e) organic acidemias