Lecture 4: Amino Acid Degredation Flashcards
Products from removal of amino group from AA
Alpha-keto acid and Ammonia
Transamination
Transferring alpha amino group to alpha ketoglutarate (most common N acceptor)
Transamination product
Glutamate
Transamination enzyme
Aminotransferase
Deamination
Releases ammonia from glutamate to liver
Deamination Enzyme
Glutamate Dehydrogenase
Urea formation rate limiting step
Carbamoyl Phosphate Synthetase 1
Urea formation in Liver equation
CO2 + Free Ammonia + H2O ——> Carbamoyl Phosphate
Where do the nitrogen’s come from in urea production?
Aspartate and ammonia
Step 2 of Urea production in liver
Carbamoyl Phosphate ——> L-Citruline
Final step of urea production
L-Arginine ——> L-Ornithine
Releases Urea
Arginase
L-Arginine ——> L-Ornithine
What enzyme breaks down extra urea in the intestine?
Bacterial Urease
What do Glucogenic AA’s produce?
Pyruvate
What do Ketogenic AA’s produce?
Acetoacetate (or precursors)
Phenylketonuria (PKU)
Excessive Phenylalanine
Not enough Phenylalanine Hydroxylase
Phenylalanine ——> Tyrosine
Phenylalanine Hydroxylase
Lack of _____ causes albinism by blocking melanin formation
Tyrosinase
_______ Produced in liver and erythrocyte producing cells of bone marrow
Porphyrins
ALAS1 inhibited by
Heme overpresence (negative feedback loop)
Lead inhibits
Aminolevulunic Acid Dehydratase
Lead also inhibits
Ferrochelatase
Heme ——> Biliverdin (step 1 Heme degradation) enzyme is ____
Heme Oxygenase
Biliverdin to Bilirubin (heme degradation step 2) enzyme is ______
Biliverdin Reductase