The goal of the PDHc-catalyzed reaction is to turn ____________ into
____________. To do this, it requires 5 other things: ____________,
_____________, ____________, ____________, and __________. Answer -
pyruvate; acetyl-CoA and NADH; TPP (thiamine, a.k.a. Vitamin B1); lipoic acid;
CoASH; FAD; NAD+ (tender loving care for Nancy);
The E1 component of PDH catalyzes the conversion of ________________ into
hydroxyethyl-TPP. The E2 component catalyzes its conversion into
_____________ via lipolysine as a intermediate aceyl carrier, leaving a reduced
lipolysine. The E3 complex then uses ________ to re-oxidize the lipolysine, and
then it also uses __________ to re-oxidize the __________ back to
___________, producing ______________. Answer - pyruvate; acetyl-CoA;
FAD; NAD+; FADH2; FAD; NADH
PDH is mainly activated by _______________ via the ______________ enzyme,
as well as by ____________ in adipocytes and ____________ in cardiac
muscle.
PDH is mainly inhibited by _______________ via the ______________ enzyme,
as well as by an excess of __________ or ___________. Answer -
dephosphorylation; PDH phosphatase; insulin; catecholamines;
phosphorylation; PDH kinase; acetyl-CoA; NADH
PDH phosphatase is _______________ by calcium and magnesium. Presence of
calcium and magnesium would therefore _________ the action of PDH itself.
,PDH kinase is _______________ by calcium among other things, and is
__________ by NADH, ATP, and acetyl-CoA. Presence of calcium would
therefore _________ the action of PDH itself, while presence of NADH would
___________ the action of PDH itself. Answer - activated; activate; inhibited;
activated; activate; inhibit
_____________________ manifests within the 1st year, showing signs of
severe metabolic acidosis, microcephaly, lethargy, hypotonia, and seizures. It is
an X-linked recessive defect in PDH, but the __________ gene is always
inactivated in females for some reason, resulting in roughly __________
manifestation between males/females. Answer - X-linked Leigh syndrome; wild
type; equal
This element is highly toxic because it inhibits dehydrogenases such as PDH,
resulting in hypoglycemia. Additionally, its pentavalent form replaces
_____________. Answer - arsenic; phosphate
The TCA cycle has no direct hormonal regulation. Instead, its rate limiting step -
catalyzed by ________________ - is inhibited by ____________ and
____________ which indicate that we have plenty of energy. Malate DH and 2-
oxoglutarate (α-ketoglutarate) DH also make ____________ and are inhibited
by it.
It requires ____________ for the reactions catalyzed by PDH and OGDH.
___________ and _________ are enzymes in the TCA cycle that are also tumor
suppressor genes. Answer - IDH (isocitrate dehydrogenase); ATP; NADH;
NADH; thiamine; fumarase; IDH (isocitrate dehydrogenase);
Some important intermediates in the TCA cycle:
___________ which is important for AA synthesis.
, ___________ which is important for gluconeogenesis
___________ which is important for heme synthesis Answer - OAA; malate;
succinyl-CoA
Overall, the TCA cycle makes 1 _______, 1 ______3 _______, and 2 _______. It
does this _______ per glucose molecule. Answer - GTP; FADH2; NADH; CO2;
twice
The _________ membrane of mitochondria is essentially impermeable,
requiring lots of transporter. The _________ membrane has lots of porins
which allow essentially free passage.
One very important antiporter is for ________ and ________, the second of
which is a regulator of respiration. Answer - inner; outer; ATP, ADP
While mitochondrial diseases are inherited almost exclusively maternally, many
mitochondrial problems have to do with nuclear DNA. Why? Answer - mtDNA
encodes 13 proteins, 2 rRNAs, and 22tRNAs, but mitochondria require over 900
proteins
The ETC is most easily inhibited at complex ______. A very potent inhibiter of
this complex is ___________ which binds tightly to ferric iron. Inhibition of this
complex stops reduction of ____________ and will shut down the ETC by
backing it up.
This poisoning can be reversed by administering hydroxocobalmin which steals
the inhibitor or by nithiodote which makes more _____________ which will
steal it as well.
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