Ox Phos Agbas Flashcards
what is the outer membrane permeable/impermeable to?
permeable to small molecules
what happens in the mitochondrial matrix?
high or low pH?
PDH
urea cycle
Fatty acid beta-oxidation
TCA enzymes
high pH
in a Redox reaction, where is the oxidant located?
on the same side as the electrons
high E0’ means what?
High affinity for electrons
BADLY wants electron pair
what enzyme catalyzes superoxide anion to hydrogen peroxide?
O2- –> H2O2
superoxide dismutase
what enzyme catalyzes hydrogen peroxide to water?
H2O2 –> H2O
catalase
what enzyme catalyzes H2O2–>GSSG?
glutathione peroxidase
what does a successful OxPhos accomplish?
transfer e- from NADH and FADH2 to O2
establish a proton gradient across the inner mitochondrial membrane
synthesize ATP
what is complex I of ETC?
action?
NADH dehydrogenase
NADH –> NAD+
donate 2e- to coQ
pumps 4H+ from matrix into IM space
complex II ETC?
action?
succinate dehydrogenase
FADH2 –> FADH
transfers 2e- from FADH2 to coQ
CoQ ETC?
action
cytochrome c reductase
accepts 2 e- from complex 1 and 2, transfer to complex 3
Complex III ETC?
function
cytochrome-c reductase
accepts 2 e- and donates them to cyto-c
pumps 4H+ from matrix into IM space
cyto C ETC?
action?
accepts e- from complex 3, donates them to 4
complex IV ETC?
action?
cytochrome c-oxidase
pumps 2 H+ into IM space
accepts e- from cyto-c and transfers them to O2, which forms water
O2–>H2O
complex V ETC?
action?
ATP synthase
moves protons from IM space into matrix to make ATP
delta G0’ = ?
-nF delta E0’
what drugs inhibit complexes 1-5?
1-RAMP 2-M 3-A 4-CCH 5-O
what happens when transfer of electrons is inhibited?
dec. in proton gradient
inhibit ATP synthesis
what complex does Kearns-Sayre syndrome affect?
Sx?
All
eye pain, ptosis, cardiac stuff, before age 20
what complex does MELAS syndrome affect?
Sx?
1, 4
m. weakness and pain, vommiting, seizures, no appetite, onset infancy
what complex does LHON syndrome affect?
Sx?
1, 3
optic nerve fucked, loss of central vision
what complex does Leigh syndrome affect?
Sx?
1, 4
dysphagia, weak motor skills, lesions in ganglia
what complex does MERRF syndrome affect?
Sx?
1, 4
muscle spasms, hearing loss, dementia
what complex does hypertrophic cardiomyopathy syndrome affect?
Sx?
3, 4
enlargement and degeneration of cardiac m.
what complex does Pearson syndrome affect?
Sx?
ALL
anemia, pancreas dysfunction
what complex does Hypertension and high cholesterol syndrome affect?
Sx?
1, 4
BP > 140/90
cholesterol > 200mg/dL
what complex does diabetes and deafness syndrome affect?
Sx?
1, 4
you know these
what contributes O2 for ROS production?
complex 1 and 3
SOD2 works where with what metal?
matrix
Manganese
SOD1 works where with what metal?
IM space
Cu and Zn
where does the Malate-Aspartate shuttle operate?
what does it do?
heart, liver, kidney
KLS –> KLH
helps NADH enter ETC at complex 1
where does the Glycerophosphate shuttle operate?
function?
Brain and skeletal m.
Bad Manner
helps FADH2 donate e- to CoQ
Lower E0’ means what?
lower affinity for e-
wants to DONATE them
what factors constitute a proton motive force (pmf)?
pH gradient
membrane potential
what is the inner membrane permeable to?
high or low pH in intermembrane space?
impermeable to small molecules except H20, CO2, O2, NH3
low pH
what factors inhibit complex 5 (ATP synthase) by uncouplers (heat generators)?
membrane damage - AraC, AZT
proton carriers - DNP, aspirin
proton channel - UCP-1
What does Adrenodoxin (FDX1) do?
participates in biosynthesis of steroid hormones in adrenal glands, metabolism of Vit D and bile acids
what does FDX2 do?
biosynthesis of heme-a (cyto-oxidase) and aconitase
what are ferredoxins?
important for what?
cyto P-450 systems that have Fe and Su
bacterial metabolism and plant photosynthesis
which ETC complex does NOT have mtDNA?
what do defects in mtDNA lead to?
II
ETC defect, MIDgut cancer, proto-oncogene damage
what does Rotenone inhibit?
treatment?
link to what disease?
complex 1
Vit K3 or IV of glucose
Parkinson’s
What does cyanide bind to?
Result?
Treatment?
Fe3+ of heme at complex 4
asphyxiation and death, Azide (N3-) also does this
thiosulfate
What does CO and NO do to ETC?
compete w/O2 for heme in complex 4
CN and H2S are noncomp inhibitor, CO is comp. inhibitor
what causes hyperthemia by disrupting the proton gradient across the IM membrane?
what is the treatment?
aspirin and DNP
gastric lavage and hemodialysis
where is the CyP family located?
act as what?
ER and mitochondria
mono-oxidases that reduce O2 to H2O
what is activated in O2 starvation?
what does this do?
IF1
stops ATP synthase from hydrolyzing ATP