Dietary protein and nitrogen

Question 1

Explain how zymogens are converted to enzymes?

Answer 1

In the stomach, HCl turns pepsin into pepsinogen,
- Trypsinogen -> trypsin by enteropeptidase on the luminal

Question 2

PKU

Answer 2

Phe to Tyr, catalyzed by phenylalanine hydroxylase
- Makes Tyr an essential amino acid
Mental retardation

Question 3

MSUD

Answer 3

Deficiency in branched chain a-ketoacid dehydrogenase (BCKD)
- Limit branched chain amino acid/ protein overall

Question 4

Alpha-1 antitrypsin deficiency

Answer 4

Leads to liver and lung disease
- Lack of inhibition of elastase in neutrophils

Question 5

Hartnup disorder

Answer 5

Lack of transport of neutral AA in renal and, in a lesser extent, intestinal cells.
- tryptophan deficiency - VIT B3; resembles Pellagra (3 Ds)

Question 6

Cystinuria

Answer 6

Disorder of proximal tubulue to reabsorb cystine