Nitrogen Flashcards
Transamination Info
Process of removing one NH3 from one amino acid and put on another with creation of alpha keto acid.
Cofactor = PLP (Vit B6); schiff base: enzyme’s Lys episilon - NH2 binds to carbon of PLP aldehyde group -> DBond
Transamination usual
Glu, Ala, Asp usually used. Glu to alpha-ketoglutarate, Ala to pyruvate, Asp to OAA
Reversible and active in liver, Liver dmg look for ALT and AST.
AA1 and a-keto2 to AA2 and a-keto1
Deamination
Release free NH3.
Enzyme = Glutamate Dehydrogenase (Glu is only AA that accepts free NH2)
Glu =(NADh and Nh3) a-ketoglutarate
NADH = ATP synth; NADPH = biosynth
Toxic NH3
needs to be removed
number 1 source = Gln; enzyme = glutaminase
removed via urea cycle
Step 1 Urea cycle
In mito
Free NH3 and HCO3 to carbomyl phosphate
Enz= carbomyl phosphate synthase 1, rqrs 2 ATP
Allosteric activation by N-acetyl-glutamate (act by Arg)
Step 2 Urea Cycle
Carbomyl phosphate and Ornithine to Citrulline
Enz = Ornithine Transcarbomylase (OTC)
OTC def
Hyperammonemia, Orotic acid build up, loss of ATP (due to diversion of alpha ketoglutarate from TCA cycle in order to soak up NH3, Glu to Gln via glutamine synthetase requires ATP so more lost)
Hepaticencephalopathy
Step 3 Urea cycle
Citrulline leaves mito to cytosol
Citrulline and Asp to Arginosuccinate
Asp provides second NH3
Enz = Arginosuccinate synthetase, rqrs ATP
Urea Cycle energy rqr
4 ATP equivalents
Urea Cycle Step 4
Arginosuccinate to Arg and Fumarate
Enzyme = arginosuccinate lyase
Some Arg used for feedback to CPS1, Fumarate to TCA cycle via conversion to malate
Urea cycle Step 5
Arg to Ornithine and Urea
Enz = Arginase
urea to urine, ornithine to mito for reuse
BUN
Liver dmg = decreased BUN
Kidney dmg = increased BUN
Tx = alternate N extract = Benzoic acid, phenylacetate, phenylbutyrate. Conjugates with gly and gln to make H20 soluble
Draw Urea Cycle
On paper
Nitrogen after meal stomach
pepsin breaks up some proteins to AA
Nitrogen after meal SI
Trypsin, chymotrypsin, elastase, carboxy peptides A and B finish job. Breaks to AA or di/tripeptide AA