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Biochem-AA Metabol

Biochem-Amino Acid Metabolism and Urea Cycle-WVSOM Class of 2012

why can't you measure uncongjugated bilirubin directly? it is still attached to albumin
what are the 10 essential amino acids? PVTTIMHALL (Phenylalanine, Valine, Tryptophan, Threonine, Isoleucine, Methionine Histidine, Arginine, Lysine, Leucine)
what are the only possiuble sources of essential amino acids? protein turnover and diet
what are some products of amino acids? purines, pyrimidines, porphyrins, and neurotransmitters
what is a positive or negative nitrogen balance? input>output; output>input
tyrosine is synthesized from what essential amino acid? phenylalanine
what is an example of a porphyrin? heme
carbon portions of amino acid degradation can be used to make what? fatty acids, ketones, glucose
alanine, aspartate, and glutamate are made from transamination of what? pyruvate, oxaloacetate, and a-ketoglutarate
where is arginine created? in the urea cycle
where does cysteine get its sulfur from? methionine
what are the 3 cofactors required for amino acid synthesis? pyridoxal phosphate (PLP), tetrahydrofolate, and tetrahydrobiopterin
what is pyridoxal phosphate (PLP) made from? B6
in what 2 ways is nitrogen removed from amino acids? deamination and transamination
what is the main difference between deamination and transamination? deamination produces ammonia
aminotransferases are also called what? what do most aminotransferases require as a cofactor? transaminases; pyridoxal phosphate (PLP)
what are the roles of alpha-ketoglutarate and glutamate in transamination? amino acid gives its amino group to alpha-ketoglutarate to produce glutamate
what are the 2 important transaminases? what are their reversible products? ALT and AST; alanine<->pyruvate and oxaloacetate<->aspartate
if liver function is impaired, what will happen to albumin? it is also impaired
what does glutamate dehydrogenase do? it deaminates glutamate to produce alpha-ketoglutarate
glutamate and an extra ammonia form what molecule? what enzyme does this? glutamine; glutamine synthase
in the liver, glutamine is broken down into glutamate and an extra ammonia how? glutaminase
aside from glutamate, what other molecule can transport ammonia to the liver? alanine
what are the roles of pyruvate and ALT in alanine transporting ammonia to the liver? pyruvate will take the ammonia from glutamate via ALT, becoming alanine; it then transports the ammonia to the liver, where it gives the ammonia back to glutamate
which amino acids are ultimately degraded to pyruvate or TCA cycle intermediates, glucogenic or ketogenic? glucogenic
which amino acids are ultimately degraded to acetyl CoA or acetoacetate, glucogenic or ketogenic? ketogenic
which hormone promotes amino acid uptake and protein synthesis? which hormone induces ubiquitin synthesis to stimulate gluconeogenesis? insulin; cortisol
which 2 hormones stimulate amino acid uptake into the liver? cortisol and glucagon
what is a state of increased body fuel usage? hypercatabolic
what enzyme converts bicarbonate and ammonia into carbamoyl phosphate? which step is this in the urea cycle? carbamoyl phosphate synthase 1; first
which enzyme converts carbamoyl phosphate and ornithine into citrulline? which step is this in the urea cycle? ornithine transcarbomylase; second
arginine is essential for what process? growth only
in the urea cycle, you need what amino acid to make what tca cycle product? aspartate; fumarate
what are the two important products of the urea cycle? arginine and fumarate
where do the nitrogens come from in the urea cycle? 1 from ammonia; 1 from aspartate
in the urea cycle, what molecule gets regenerated? ornithine
urea cycle regulation is primarily what kind of loop? feedforward
what synthesizes n-acetylglutamate (NAG)? glutamate or acetyl coa
what is n-acetylglutamate's effect on CPS1? allosteric regulator
what are the two possible fates of urea? filtered and urinated by kidney; broken down by bacteria into ammonia and put back through the cycle
what happens to BUN in kidney failure? liver failure? elevated; reduced
what is the common precursor of both ammonia and aspartate? glutamate
what is the most common urea cycle disorder? otc
otc is what kind of hereditary disease? x-linked
Created by: mhassan
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