Exam 3 Flashcards

1
Q

What is lysosomal degradation, and what is its optimal pH?

A

involves several proteases
pH= 5 and is much less active at a pH of 7 (pH of cytoplasm) bc it won’t work if it’s not in the lysosome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

How does lysosomal degradation differ in well-nourished versus starving cells?

A

In well-nourished cells, lysosomal degradation is nonselective

In starving cells, selective protein degradation occcurs, especially in organs like the liver and kidney (NOT brain)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

When one is in starvation what sequence mediates it in lysosomal degradation?

A

Mediated via KEFRQ sequence in proteins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Where is lysosomal activity increased?

A
  • Muscle wasting
    (use it or lose it- lack of use, injury)

*arthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Draw PLP

A

lec 8; slide 11

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the two stages in which transamination rxns occur

A

Ala + 𝛼-KG↔︎ pyruvate + Glu

stage 1
Ala +PLP↔︎ pyruvate + pyridoxiamine-P

stage 2
pyridoxamine-P + 𝛼-KG↔︎ Glu+ PLP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are some transamination rxns (AND DRAW):
Alanine↔︎
Aspartic acid↔︎
Glutamic acid↔︎

A

Alanine↔︎ pyruvate
Aspartic acid↔︎ OXAC
Glutamic acid↔︎ 𝛼-KG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what is the difference between transamination, tautomerization, and hydrolysis

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Glutamate DH

A

removes NH4⁺ by oxidate deamination

✿ Glu+ NAD(P)⁺ ↔︎ ⍺-KG + NH4⁺ + NAD(P)H

✿ reversible
✿High Km for Glu

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

draw structure of urea

A

H₂N-C=O-NH₂
lec8, slide 25

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Draw the urea cycle

A

lec 8, slide 26

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Draw reaction of carbamoyl phosphate synthase I

A

2 ATP+ HCO3⁻ + NH3→ carbamoyl P + 2ADP + 2Pi

CPSII is involved in N.A metabolism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Rate limiting step for urea cycle?

A

Carbamoyl Phosphate Synthase I

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Ornithine transcarboxylase

A

carbamoyl P + ornithine →
citruline

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Mutations which reduce efficiency of urea cyle

A

Hyperamonemia
-lethargy
-retardation
✬Treat w/ Δ diet, NAG analogs, ↑Arg (→ornithine)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Serine —> pyruvate through??

Is there a pyridoxamine phosphate intermediate?

Is PLP involved?

A

Serine Dehydratase

No

Yes

17
Q

What AA go directly into TCA cycle?

A

❁Alanine
❁Cysteine
❁Glycine
❁Serine
❁Threonine
❁Tryptophan
===pyruvate

18
Q

Which of the following is a transamination? Which of the following requires VitB6?
Phe→ Phenylpyruvate

Phe→ Tyrosine

A

Phe→ Phenylpyruvate (it involves ⍺KG↷ Glu) same

19
Q

The normal pathway for phenylalanine is….?

what happens if there is a deficiency in the enzyme?

A

Phe (Phe Hydroxylase) tyrosine
➡︎

deficiency: turns into phenylpyruvate(transamination) and an accumulation of phenyl ketones ocurr.

This causes PKU

20
Q

What is the solution to PKU?

A

↓↓↓ Phe diet