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1
Q

what is the outer membrane permeable/impermeable to?

A

permeable to small molecules

2
Q

what happens in the mitochondrial matrix?

high or low pH?

A

PDH
urea cycle
Fatty acid beta-oxidation
TCA enzymes

high pH

3
Q

in a Redox reaction, where is the oxidant located?

A

on the same side as the electrons

4
Q

high E0’ means what?

A

High affinity for electrons

BADLY wants electron pair

5
Q

what enzyme catalyzes superoxide anion to hydrogen peroxide?

O2- –> H2O2

A

superoxide dismutase

6
Q

what enzyme catalyzes hydrogen peroxide to water?

H2O2 –> H2O

A

catalase

7
Q

what enzyme catalyzes H2O2–>GSSG?

A

glutathione peroxidase

8
Q

what does a successful OxPhos accomplish?

A

transfer e- from NADH and FADH2 to O2

establish a proton gradient across the inner mitochondrial membrane

synthesize ATP

9
Q

what is complex I of ETC?

action?

A

NADH dehydrogenase

NADH –> NAD+

donate 2e- to coQ
pumps 4H+ from matrix into IM space

10
Q

complex II ETC?

action?

A

succinate dehydrogenase

FADH2 –> FADH

transfers 2e- from FADH2 to coQ

11
Q

CoQ ETC?

action

A

cytochrome c reductase

accepts 2 e- from complex 1 and 2, transfer to complex 3

12
Q

Complex III ETC?

function

A

cytochrome-c reductase

accepts 2 e- and donates them to cyto-c

pumps 4H+ from matrix into IM space

13
Q

cyto C ETC?

action?

A

accepts e- from complex 3, donates them to 4

14
Q

complex IV ETC?

action?

A

cytochrome c-oxidase
pumps 2 H+ into IM space

accepts e- from cyto-c and transfers them to O2, which forms water
O2–>H2O

15
Q

complex V ETC?

action?

A

ATP synthase

moves protons from IM space into matrix to make ATP

16
Q

delta G0’ = ?

A

-nF delta E0’

17
Q

what drugs inhibit complexes 1-5?

A
1-RAMP
2-M
3-A
4-CCH
5-O
18
Q

what happens when transfer of electrons is inhibited?

A

dec. in proton gradient

inhibit ATP synthesis

19
Q

what complex does Kearns-Sayre syndrome affect?

Sx?

A

All

eye pain, ptosis, cardiac stuff, before age 20

20
Q

what complex does MELAS syndrome affect?

Sx?

A

1, 4

m. weakness and pain, vommiting, seizures, no appetite, onset infancy

21
Q

what complex does LHON syndrome affect?

Sx?

A

1, 3

optic nerve fucked, loss of central vision

22
Q

what complex does Leigh syndrome affect?

Sx?

A

1, 4

dysphagia, weak motor skills, lesions in ganglia

23
Q

what complex does MERRF syndrome affect?

Sx?

A

1, 4

muscle spasms, hearing loss, dementia

24
Q

what complex does hypertrophic cardiomyopathy syndrome affect?

Sx?

A

3, 4

enlargement and degeneration of cardiac m.

25
Q

what complex does Pearson syndrome affect?

Sx?

A

ALL

anemia, pancreas dysfunction

26
Q

what complex does Hypertension and high cholesterol syndrome affect?

Sx?

A

1, 4

BP > 140/90
cholesterol > 200mg/dL

27
Q

what complex does diabetes and deafness syndrome affect?

Sx?

A

1, 4

you know these

28
Q

what contributes O2 for ROS production?

A

complex 1 and 3

29
Q

SOD2 works where with what metal?

A

matrix

Manganese

30
Q

SOD1 works where with what metal?

A

IM space

Cu and Zn

31
Q

where does the Malate-Aspartate shuttle operate?

what does it do?

A

heart, liver, kidney
KLS –> KLH

helps NADH enter ETC at complex 1

32
Q

where does the Glycerophosphate shuttle operate?

function?

A

Brain and skeletal m.
Bad Manner

helps FADH2 donate e- to CoQ

33
Q

Lower E0’ means what?

A

lower affinity for e-

wants to DONATE them

34
Q

what factors constitute a proton motive force (pmf)?

A

pH gradient

membrane potential

35
Q

what is the inner membrane permeable to?

high or low pH in intermembrane space?

A

impermeable to small molecules except H20, CO2, O2, NH3

low pH

36
Q

what factors inhibit complex 5 (ATP synthase) by uncouplers (heat generators)?

A

membrane damage - AraC, AZT
proton carriers - DNP, aspirin
proton channel - UCP-1

37
Q

What does Adrenodoxin (FDX1) do?

A

participates in biosynthesis of steroid hormones in adrenal glands, metabolism of Vit D and bile acids

38
Q

what does FDX2 do?

A

biosynthesis of heme-a (cyto-oxidase) and aconitase

39
Q

what are ferredoxins?

important for what?

A

cyto P-450 systems that have Fe and Su

bacterial metabolism and plant photosynthesis

40
Q

which ETC complex does NOT have mtDNA?

what do defects in mtDNA lead to?

A

II

ETC defect, MIDgut cancer, proto-oncogene damage

41
Q

what does Rotenone inhibit?

treatment?

link to what disease?

A

complex 1

Vit K3 or IV of glucose

Parkinson’s

42
Q

What does cyanide bind to?
Result?
Treatment?

A

Fe3+ of heme at complex 4

asphyxiation and death, Azide (N3-) also does this

thiosulfate

43
Q

What does CO and NO do to ETC?

A

compete w/O2 for heme in complex 4

CN and H2S are noncomp inhibitor, CO is comp. inhibitor

44
Q

what causes hyperthemia by disrupting the proton gradient across the IM membrane?

what is the treatment?

A

aspirin and DNP

gastric lavage and hemodialysis

45
Q

where is the CyP family located?

act as what?

A

ER and mitochondria

mono-oxidases that reduce O2 to H2O

46
Q

what is activated in O2 starvation?

what does this do?

A

IF1

stops ATP synthase from hydrolyzing ATP