What is the 1st step in Urea Synthesis of the Urea Cycle
What is the 2nd step in Urea Synthesis of the Urea Cycle
What is the 3rdt step in Urea Synthesis of the Urea Cycle
What is the 4th step in Urea Synthesis of the Urea Cycle
What are the final products of the Urea Cycle
Urea & Ornithine
Where does Urea Synthesis take place
Where is Carbamoyl phosphate made
Where is the majority of the Urea cycle take place
Amino acid nitrogen is carried to the liver by which two Amino Acids
Alanine & Glutamine.
Type I hyperammonemia (CPSI or N-acetylglutamate synthase deficiency) prevents what from being made in the Urea Cycle
Type II hyperammonemia (ornithine transcarbamoylase (X-linked recessive)) prevents what from being made in the Urea Cycle
Citrullinuria Type I (argininosuccinate synthetase deficiency) prevents what from being made in the Urea Cycle
Argininosuccinic acidemia (argininosuccinate lyase deficiency) prevents what from being made in the Urea Cycle
Fumarate (TCA Cycle) & Arginine
Argininemia (arginase deficiency) prevents what from being made in the Urea Cycle
Urea & Ornithine.
Hyperornithinemia-Hyperammonemia-Homocitrullinuria (HHH) syndrome (ornithine translocase deficiency, the enzyme that transports ornithine into the mitochondria)
Carbamoyl phosphotase and Ornithine Reaction.
This autosomal recessive disease is defective absorption of what 4 AAs which accumulate in the urine. High levels of cystine can cause cystine stones in kidneys,ureters and bladder
Cystinuria (COAL) Cystine, Ornithine, Arginine and Lysine, in intestinal and proximal tubule kidney epithelial cells.
What is the disease that is defective transport of neutral amino acids (including tryptophan) into intestinal and kidney epithelial cells, causes Neutral amino acids accumulate in urine, & pellagra-like symptoms can
develop (skin rash, ataxia, tremors)
What are the 2 ways proteins are degraded
Extracellular (Lysosomal Degradation) & Intracellular (Ubiquitin-mediated proteasomal degradation)
What is the term for the removal of Amino Acid Nitrogen
Amino Acid Nitrogen is generally collected on what amino acid
Is Transamination Reversible or Irreversible
Reversible. Transamination is involved in both the synthesis & degradation of the amino acids.
Which 2 Amino Acids cannot participate in Transamination
Lysine & Threonine.
What catalyzes the Transamination reactions
What is the Coenzyme in Transamination
pyridoxal phosphate (PLP) which we get from Vitamin B6 (pyridoxine).
What enzyme coupled with transamination reactions is important for both the degradation and synthesis of amino acids
Glutamate dehydrogenase (GDH)
The oxidative deamination (Disposal of AA) reaction requires what coenzyme
The reductive amination (Synthesis of AA) reaction requires what coenzyme
What 3 enzymes "fix" free ammonia into organic molecules
Glutamate dehydrogenase, Glutamine synthetase, Carbamoyl phosphate synthetase (1st step in Urea Synthesis).
What is the main detoxification reaction of Ammonia in the brain