What is the role of HCl in protein digestion?
Denatures proteins and activates pepsinogen to pepsin.
What is the role of pepsin in protein digestion?
Endopeptidase that breaks proteins into peptides.
What pancreatic enzymes digest proteins?
Trypsin, chymotrypsin, elastase, and carboxypeptidases.
What pancreatic zymogens are involved in protein digestion?
Trypsinogen, chymotrypsinogen, proelastase, procarboxypeptidase A and B.
Role of glutamine in ammonia transport?
Transports ammonia safely from tissues to liver and kidney.
Role of alanine in ammonia transport?
Transports ammonia from muscle to liver.
What is transamination?
Transfer of amino group from an amino acid to a keto acid.
What is deamination?
Removal of amino group as free ammonia (NH₃).
What are the most common transaminases?
ALT and AST.
What does elevated ALT and AST indicate clinically?
Liver cell damage (e.g., hepatitis, cirrhosis).
What is the glucose-alanine cycle?
Cycle where alanine transports nitrogen from muscle to liver and glucose returns to muscle.
What is nitrogen balance?
Difference between nitrogen intake and nitrogen excretion.
What is positive nitrogen balance?
Intake exceeds loss (growth, pregnancy).
What is negative nitrogen balance?
Loss exceeds intake (starvation, illness).
Where does the urea cycle occur?
In the liver.
Subcellular location of the urea cycle?
First two steps in mitochondria, remaining steps in cytosol.
Rate-limiting enzyme of the urea cycle?
Carbamoyl phosphate synthetase I (CPS I).
Why is the urea pathway called a cycle?
Ornithine is regenerated and reused.
What activates CPS I?
N-acetylglutamate.
When does urea cycle activity increase?
High protein intake and starvation.
How is ammonia toxic to the brain?
Causes glutamine accumulation, cerebral edema, and ATP depletion.
Causes of congenital hyperammonemia?
Urea cycle enzyme deficiencies.
Causes of acquired hyperammonemia?
Liver failure, cirrhosis, hepatitis, drugs (e.g., valproate).
General features of congenital hyperammonemia?
Vomiting, lethargy, poor feeding, developmental delay, high ammonia.