Biochem lec 5A - important questions Flashcards
first phase of amino acid catabolism
-removal of the alpha-amino group forming ammonia and the corresponding alpa-keto acid (the cabron skeletons of aa)
-a portion of free ammonia is excreted in uring, but most is used in the synthesis of urea, which is quantitavley the most important route for disposing nitrogen from the body
what is the first step in catabolism of most AA
transamination - transfer of their a-amino group to a-ketoglutarate
products of transamination
a-keto acid and glutamate
what is the most common N acceptor
a-ketoglutarate
what is transamination catalzyed by
aminotransferase
(transfer of amino group from one carbon skeleton to another)
all aa participate in transamination in their catbaolims except
lysine and threonine (deamination instead)
deamination def
deaminatino results in the libteration of the amino group as free ammonia
what is deamination of glutamate to a-ketoglutarate catalzyed by
glutamate dehydrogenase
what is the major disposal form of amino group derived from amino acids, and accounts for 90% of N components in urine
urea
urea is produced where and tranported how to where for excretion in urine
produced by liver, transported in blood to kidneys
what does the urea cycle convert and why
ammonia to urea (less toxic)
what is the rate limiting step in urea cycle
carbamoyl phosphate synthetase I
what is an essential activator of carbamoyl phosphate synthetase I
N-acetylglutamate
glucogenic AA
whose catabolism yields pyruvate or one of the intermedaites of the TCA cycle
ketogenic AA
whose catbolism yields either acetoacetate or one of its precursors (acetly CoA or acetoacetly CoA)
phenylketonuria (PKU)
-results in overaccumulation of phenylalanine, due to deficiency of phenylalanine hydroxylase
-strict diet control (to reduce phe) can prevent mental retardation and other brain damage
albinism reults from what
results from lack of tyrosinase, and thus melanin is not produced
albinos get skin cancer easily
what is the major site of heme biosynthesis
liver and erythrocyte-producing cells of bone marrow (bone marrow accounds for about 85%)
initial reaction and the last 3 steps in the formation of porphyrins occur where, and where do the intermediate steps occur
intermediate steps in the cytosol
last 3 steps in the mitochondria
why are mature rbcs unable to synthesize heme
lack mitochondria
degradation of heme (2 steps)
heme converted to biliverdin and CO by heme oxygenase
biliverdin converted to bilirubin by biliverdin reductase
enzyme for heme to biliverdin
heme oxygenase
enzyme for biliverdin to bilirubin
biliverdin reductase
bilirubin characteristics
very toxic and insoluable
jaundice (iterus)
yellow color of skin, nail beds and sclerae (white of eyes) - causes by deposition of bilirubin, secondary to increased bilirubin levels in the blood (hyperbilirubinemia)
norepinephrine and epinephrine function
-regulate carbohydrate and lipid metabolism
-involved in fight or flight response
parkinson disease due to what
due to insufficient dopamine production caused by the loss of dopamine-producing cells in the brain (neurodegenerative movement disorder)
catecholamines are inactived by deamination using what as the methly donor
SAM