Exam 2 Peng 4

Question 1

degradation of aromatic rings require?

Answer 1

strong enzymes like oxygenases

Question 2

phenylalanine and tyrosine produce?

Answer 2

fumarate

Question 3

phenylalanine is converted to tyrosine how?

Answer 3

a MONOoxygenase called phenylalanine hydroxylase

Question 4

tyrosine degradation produces?

Answer 4

alanine, fumerate and acetoacetate

Question 5

bracnhed cain AA are degradeed to

Answer 5

acetyl CoA, acetoacetate, propionyl CoA

Question 6

Lecuine
valine
isoleucine (all converted to?)

Answer 6

leucine –> acetoacetate + Acetyl CoA
valine –> CO2 + propionyl CoA
isoleucine –> acetyl CoA _ propionyl CoA

Question 7

branched chain AA are metabolized primarily in?

Answer 7

peripheral tissue like muscle

Question 8

what is oxidative decarboxylation?

Answer 8

removal of carboxyl group via branched chain alpha keto acid dehydrogenase

Question 9

alanine, asparate and glutamineare synthesized by?

Answer 9

reversed transamination

• pyruvate= alanine
• oxyaloacetate= aspartate
• alpha-ketoglutarate= glutamate

Question 10

serine to glycine

glycine to serine?

Answer 10

serine to glycine via methyl THF

glycine to serine via THF

Question 11

glutamine is synthesized by what two things?

Answer 11

reversed transamination and oxidative deamination

Question 12

what is phenylketonuria?

Answer 12

deficiency in phenylalanine

• ** most common inborn AA error in metabolism
• effects CNA
• treat diet

Question 13

what is albinsim?

Answer 13

defects in thrysine metabolism, tyrosinase

• deficiency in production of melanin
• hypopigmentation, photophobia, elevated risk of skin cancer

Question 14

what is maple syrup urine disorder?

Answer 14

deficiency in mitochondrial BCKD

• increased alpha- keto acids that cause BRAIN damage
• control by deit, super hard to control bc you still need essential AA