protein targeting and export 2 Flashcards

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1
Q

to the nucleolus

how does it go there and what is stopping it

A

not membrane bound
NOLS - nucleoar localisation signal
which is a longer version of NLS and has a run of basic residues K/R

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2
Q

going to mitochondria

what is needed and home much goes in

A

99% of mitochondrial proteins come from nuclear genome

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3
Q

mitochondrial matrix targeting what is it

what does it require

A

n terminal matrix targeting sequence

20-50 aa rich in hydrophobic aa +ve, and hydroxylated aa (s/t) forming amphipathic alpha helix

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4
Q

amphipathic alpha helix which has

A

+ve chare on one side

hydrophobic on other

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5
Q

mitochondrial targeting peptide mTP

what does it do

A

binds to receptor on mitochondrial outer surface and brought towards translocase of the outer membrane TOM

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6
Q

step 1 of translocation

A

unfolding helped by chaperones (HSC70) which requires ATP

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7
Q

step 2 of translocation

A

mTP binds to import receptor

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8
Q

step 3 of translocation

A

brings protein to TOM pore

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9
Q

step 4 of translocation

A

protein begins to pass through

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10
Q

step 5 of translocation

A

close proximity of TIM complex enables passage through inner membrane helped by ATP turnover by matrix HSC70

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11
Q

step 6 of translocation

A

mTP is removed by protease

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12
Q

step 7 of translocation

A

chaperones help refold protein to active state

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13
Q

what else foes the TIM pore/membrane do to help translocation

A

mitochondrial MP helps drive import via electrophorysis due to attraction of +vely charged AA in mTP towards matrix
dues to oxidative phosphorylation having high [h+] in the intermembrane space

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14
Q

leaky scanning what is it

A

isoforms of a protein with different N-termini can be produced by thus
if the sequence context surrounding the initiation codon is weak

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15
Q

alternate initiation

extended open reading frame eORF

A

translated from a non-AUG contains NLS

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16
Q

alternate initiation

annotated form aORF

A

translated from a weak AUG stays in cytosol

17
Q

alternate initiation

shorter truncated form tORF

A

translated from a internal AUG

contains mTP

18
Q

mitochondrial diseases
human deafness - dystonia syndrome
what causes it
what doesit cause

A

in recessive X-linked
leads to hearing loss, metal retardation and blindness
caused by mutation in TIMM8A

19
Q

what does a mutation in TIMM8A do

A

moves proteins to intermembrane space

20
Q

what does the mutation of TIM14 cause

A

autosomal recessive dilated cardio-myopathy with ataxia (DCMA) syndrome

21
Q

what can the alpha-beta peptide do to the TOM TIM pores

A

plug them up
linked to Alzheimer
and inhibition of cytochrome C oxidase