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BMS2014 - Biology of Ageing > Lecture 16 - Mitochondria in health and disease 1 > Flashcards

Lecture 16 - Mitochondria in health and disease 1 Flashcards

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1
Q

mitochondrial DNA is closed…….., locted in the mitochondrial…… and there are multiple…… per cell

A

covalently, matrix, copies

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2
Q

how many protein encoding genes are there for mitochondrial DNA? what components to they all encode?

A

13, hydrophobic OXPHOS

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3
Q

how many rRNA and how many tRNAs does mitochondrial DNA have?

A

2 r, 22 t

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4
Q

name the 4 non-OXPHOS functions of mitochondria

A

apoptosis, cell growth, cell signalling, cell differentiation

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5
Q

name the 5 functions of mitochondria

A

metabolite transport, OXPHOS assembly, mitophagy, heme biosynthesis, protein import/sorting

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6
Q

name the 4 stages of aerobic respiration

A

glycolysis, Beta-oxidation, TCA cycle, OXPHOS,

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7
Q

name the products of the TCA/krebs cycle

A

2 carbon dioxide, 3 NADH, FADH2, GTP, 2H+, CoA

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8
Q

which 2 complexes carry out beta-oxidation of fatty acids?

A

complex 3 (CoQ) and complex 1

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9
Q

what is the name of complex 1?

A

NADH ubiquinone oxidoreductase

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10
Q

in complex 1:
1) …..electrons from NADH are transferred to chlorine
2) NADH is oxidised and ….. is reduced
3) FMN is oxidised as it passes electrons to ……
4) Fe-S is oxidised as it passes electrons to ………
5) H+ are pumped across the IMM by …..

A

1 - 2
2 - FMN
3 - Fe-S
4 - ubiquinone
5 - chlorine

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11
Q

in complex 1 how many H+ is there a net transfer of from matrix to IMS?

A

4

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12
Q

what is the name of complex 2?

A

succinate ubiquinone oxidoreductase or succinate dehydrogenase

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13
Q

in complex 2 what is the function of each subunit (SDHA,B,C,D)

A

SDHA = flavoprotein
B = Fe-S protein
C = cytochrome b560
D = cytochrome b

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14
Q

which 2 subunits of complex 2 are hydrophillic and in the matrix?

A

A, B

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15
Q

in complex 2, which 2 subunits are hydrophobic and integral membrane proteins?

A

C, D

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16
Q

complex 2 in the ETC catalyses the oxidation of succinate to what?

A

fumarate

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17
Q

in complex 2, electrons from FADH2 are transferred to ubiquinone via the Fe-S clusters in which subunit, reducing it to ubiquinol?

A

SDHB

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18
Q

what is the name of complex 3?

A

ubiquinol cytochrome c oxidoreductase

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19
Q

each monomer of complex 3 is made of how many polypeptide subunits?

A

11 each monomer

20
Q

name the 4 active redox prosthetic groups in complex 3

A

2x b-type cytochromes (bL and bH)
cytochrome c1
rieske protein (Fe-S cluster)

21
Q

the Q cycle in complex 3 results in:
- the oxidation of ubiquinol (QH2) to
……… (Q)
- the reduction of cytochrome c (cyt
c3+ to cyt c…….+)
- the concomitant transfer of four
protons into the intermembrane
space, via a two-cycle process
involving a ………
intermediate

A

ubiquinone, 2+, semiquinone

22
Q

what is the name of complex 4?

A

cytochrome c oxidase

23
Q

in complex 4, subunits 1-3 are …… encoded and subunits 4-13 are…… encoded

A

mtDNA, nuclear

24
Q

complex 4 contains how many each of non-covalently bonded haem a and copper centres? what are they used for?

A

2 of each, for electron transfer

25
Q

which haem a and which copper centre in complex 4 form a binuclear centre, binding to oxygen to reduce it?

A

CuB and cytochrome a3 (haem)

26
Q

which complex in the ETC is the terminal electron acceptor, reducing oxygen to water and its reaction results in proton pumping?

A

complex 4

27
Q

name the 2 domains of complex 5 (ATP synthase)

A

F0 and F1

28
Q

which domain of complex 5/ATP synthase contain a proton channel?

A

F0

29
Q

which domain of complex 5/ATP synthase contains C ring subunits?

A

F0

30
Q

which domain of complex 5/ATP synthase contains additional subunits ATP6, ATP8, DAPIT?

A

F0

31
Q

which domain of complex 5/ATP synthase contains 3alpha and 3beta subunits?

A

F1

32
Q

which domain of complex 5/ATP synthase contains one gamma, one delta and one epsilon subunit, making up the central stalk?

A

F1

33
Q

which domain of complex 5/ATP synthase contains subunits b, d, F6 and OSCP which make up the peripheral stalk?

A

F1

34
Q

which domain of complex 5/ATP synthase contains an active site for ATP synthesis?

A

F1

35
Q

which domain of complex 5/ATP synthase protrudes into the matrix and which domain is embedded in the inner membrane?

A

F1 protrudes
F0 embedded

36
Q

which ring rotates first in ATP synthase, causing the gamma-apsilon central stalk to rotate? what does this result in in the 3xbeta subunits in the F1 domain enabling ADP +Pi = ATP?

A

C ring, results in conformational changes of the 3 beta subunits

37
Q

true or false, OXPHOS is under dual genetic control?

A

true

38
Q

name the 2 stands of mtDNA saying whether they are guanine rich or cytosine rich?

A

H strand is guanine rich
L strand is cytosine rich

39
Q

approximately how many bp is human mtDNA? what % of the human genome does it account for roughly?

A

16,569bp, about 0.0006%

40
Q

the non coding area of the mtDNA is known as what? which 2 functions occur in this region?

A

D loop, replication initiation and transcription initiation

41
Q

what are the 4 genetic rules of mtDNA?

A

polyploid genome
heteroplasmy
threshold effect
maternal inheritance

42
Q

why is the polyploid genome of mtDNA? what does it allow?

A

allows heteroplasmy and mtDNA inheritance

43
Q

what is heteroplasmy of the mtDNA?

A

means the mtDNA genome has two or more variants = normal and mutated DNA copies

44
Q

the threshold effect in mtDNA is when the number of …… mtDNA copies passes a threshold, meaning the ETC and …… fail

A

mutated, ATP synthesis

45
Q

which 2 molecules detect cells with deleterious mtDNA mutations? what colour does each turn when this activity is detected?

A

cytochrome c oxidase (brown) and SDH (blue)

46
Q

primary mtDNA disease is characterised by defective what?

A

OXPHOS

47
Q

when can primary mtDNA disease present? is there a cure? is there a wide spectrum of clinical phenotypes?

A

can present at birth, childhood or adulthood. no cure. yes

BMS2014 - Biology of Ageing (25 decks)

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