ETC Flashcards
what is the major ATP generating organelle in the cell
mitochondrion
inner membrane of mitochondria sturcture
highly invaginated, high surface area
mitochondrial matrix
internal soluble compartment of the mitochondrion, bounded by inner membrane
what is the out membrane composed of
half protein, half lipis
what is inner membrane composed of
80% protein, 20% lipid
outer membrane permeability
highly permeable
inner membrane permeability
less permeable
what does the matrix contain
soluble enzyme system, PDH complex, enzymes for fatty acid beta oxiation, some urea cycle enzymes
ETs
series of chain of electron carries in inner mitochondrial membrane that funnel electrons from reduced substrates to molecule oxygen to form water
what happes to free energy in ETS
loses free energy
oxidative phosphorylation
coupling of oxidation of metabolic substrates by ETS to phosphorylation of ADP to form ATP
What proteins are in the ETS
dehydrogenases, iron containing proteins, nonheme-iron proteins
what lipids are in the ETS
coenzyme Q
what are the dehydrogenases of the ETS
succinate dehydrogenase, NADH dehydrogenase, fatty acyl-CoA dehydrogase, glycerol 2 phosphate dehydrogenase
function of NADH dehydrogase
oxidizes NADH to NAD+
function of fatty acyl-CoA dehydrogenase
catalyzes the intiral step in beta oxidation of fatty acids
what are the iron contain proteins in ETS
cytochromes
waht is uniwue about the iron of cytochromes
undergoes fluctuation between teh reduced and oxidized state (Fe2+, Fe3+)
what are the nonheme iron proteins in ETS
they contain irson complexed to sulfide ions
complex I
NADH-CoA reductase
Comples II
succinate CoQ-reductase
Complex III
CoQH2 cytochrome c reductase
complex IV
cytochrome oxidase
what are inhbitors of complex IV
cyanide CO
which complex contains Cu that flucates between Cu2+ and Cu3+
complex IV
which complexes yield ATP
I, III, IV
the amount of energy liberated via ETS would form how many ATPs under standard conditions
7
chemiosmotic model
free energy derived from ETS direces active transport system that pumps H+ out of mitochondrial matrix, establishing electrochemical gradient
what are the sites of proton pumping in the ETS
I, III, IV
what is complex V
ATP synthase, couples the re-entry of protons to the synthesis of ATP
what is the F0 unit
multisubunit, transmembrane complex that forms a proton channel through the membrane
what is the F1 component
multisubunit structure that extends into the matrix and forms the catalytic moiety invovled in the actual synthesis of ATP from ADP+Pi
function of uncouplers of ox phos
provide an alternate froute for re-entery of protons to mitochondral matrix
examples of uncoupler of ox phos
DNP
where is brown fat
hibernating animals, newborns
what is uniwue about brown fat
contains mitochondria that have biological uncouplign protein, helps the organism stay warm
how does ATP get to cytosaol
adenine nucleotide translocase that exchanges ATP for ADP
what inhibits adenine nucleotide translocase
atractyloside
symptoms of LHON
sudden onsite blindness in young adults due to death of optic nerve
what is LHON due to
mutation in complex I
MERRF is caused by
point mutaiton in mitochondrial gene for lysin tRNA
symptoms of MERRF
myoclonus and ataxia with generalized seizures
symptoms of MELAS
muscle has ragged red ribers, but cytochrome oxidase is near normal
what is MELAS caused by
point mutation in gene for leucine tRNA
what does DNP do
uncoupling of ox phos
