Protein and AA

Question 1

Amino acids are synthesized from derivatives of what ___.

Answer 1

Glucose 6 phosphate and then are fromed by transamination into a keto acid

Question 2

Aromatic family of amino acis are synthesized from ___.

Answer 2

Ribose-5-Phosphate from the PPP

Question 3

Serine family is synthesized from___.

Answer 3

3PG from glycolysis.

Question 4

Homocystinuria?

Answer 4

autosomal recessive inherited deficiency in CBS. Symptoms manifest from B6,B12, or folate deficiencies that affects the CBS activity, THF path, or re methylation of homocysteine.

Question 5

Catabolism of BCAA’s?

Answer 5

Degraded in mm kidney and brain by transamination oxidative carboxylation and FAD linked dehydrogenation. Not degraded in liver bc there is no amino transferase.

Question 6

What are the consequences of hyperhomocysteinemia and homocystinuria?

Answer 6

They are srisk factors in atherosclerotic heart disease and stroke also eye lens dislocation, osteoperosis and mental retardation. Vitamin sup. can help in some cases.

Question 7

What causes secondary PKU?

Answer 7

Deficiency in tetrahydrobiopterin THB which is an essential cofactor of phe hydroxylase.

Question 8

Tyrosine is a precursor for what important moleucles?

Answer 8

Catecholamine neurotransmitters such as dopamine, norepinephrine and epinephrine. Also melanin pigments. Catecholamine sysnthesis requires TBH vitamin c and SAM (SAM is a major methyl donor in many reactions). (Defeciency in TBH causes secondary PKU, tyrosine is derived from Phe)

Question 9

Trp is a precursor for what important molecules?

Answer 9

Serotonin and also niacin. Serotonin can also be converted into melatonin.

Question 10

Parkinsonism?

Answer 10

Loss of concersion of dopa to dopamine in the brain due to neural tissue distruction. Treat with L dopa which can cross BBB bc dopamine itself cant cross barrier.