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Biochem-AA Metabol
Biochem-Amino Acid Metabolism and Urea Cycle-WVSOM Class of 2012
| Question | Answer |
|---|---|
| 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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