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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?
|tyrosine is synthesized from what essential amino acid?
|what is an example of a porphyrin?
|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?
|what are the 3 cofactors required for amino acid synthesis?
|pyridoxal phosphate (PLP), tetrahydrofolate, and tetrahydrobiopterin
|what is pyridoxal phosphate (PLP) made from?
|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?
|aside from glutamate, what other molecule can transport ammonia to the liver?
|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?
|which amino acids are ultimately degraded to acetyl CoA or acetoacetate, glucogenic or ketogenic?
|which hormone promotes amino acid uptake and protein synthesis? which hormone induces ubiquitin synthesis to stimulate gluconeogenesis?
|which 2 hormones stimulate amino acid uptake into the liver?
|cortisol and glucagon
|what is a state of increased body fuel usage?
|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?
|in the urea cycle, you need what amino acid to make what tca cycle product?
|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?
|urea cycle regulation is primarily what kind of loop?
|what synthesizes n-acetylglutamate (NAG)?
|glutamate or acetyl coa
|what is n-acetylglutamate's effect on CPS1?
|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?
|what is the common precursor of both ammonia and aspartate?
|what is the most common urea cycle disorder?
|otc is what kind of hereditary disease?