Mitochondrial Bioenergetics Flashcards
Synthesized from B6 (pantothenic acid)
CoA
Activated by substrates and low energy (also Ca2+ and Mg2+)
Dependent on Magnesium
Inhibited by Phosphorylation and its products and high energy (NADH and ATP)
PDH
PDC remains phosphorylated
Pyruvate turns into lactose
Lactic Acidosis
Controlled with B1 (thiamine) and b6 (biotin)
Neonatal lactic acidosis
Links SH groups on 2 lipoic acids irreversibly
Inhibits PDC
Affects alpha ketoglutarate dehydrogenase
Aresenite
Rate Limiting step in TCA
Isocitrate to alpha ketoglutarate via isocitrate dehydrogenase
Reacts with coA to form fluoroacetyl coA
Condenses with OAA to make fluorocitrate (competitive inhibitor of isonitase)
Inhibits PFK 1 and glycolysis
Rat poisoning (fluoroacetate)
Involved in heme synthesis
involves vitamin B6
Succinyl coA
Can’t convert pyruvate to OAA; Lactic acid made instead of OAA; Seizures, muscle weakness, ataxia
Pyruvate carboxylase deficiency
Rare disorder that leads to global developmental delay and severe neurological problems; also causes microcephaly, metabolic acidosis, mental retardation; stress causes acute metabolic acidosis and hypoketoic hypoglycemia
Urinary excretion of 2-oxoglutaric
2-oxoglutaric acid aciduria
Death by age 2
Stress: acute metabolic acidosis and hypoketoic hypoglycemia
Urinalysis indicates levels of fumarate, succinate, lactate, and eventual 2-oxoglutaric
Fumarase deficiency
Mild methylmalonic acidemia
Diagnosed via genetic testing
Succinyl coA cant be converted to Succinate
Succinyl CoA synthetase deficiency
Small proteins that serve as carriers for electrons in P540 systems
Clusters of iron and sulfur
Ferrodoxin
Another electron transport system that works mainly in ER and mitochondria; Transfers NADPH to heme via flavoprotein
Cytochrome P450
Forms when O2 splits in two
Free Radical
Intermediate in transfer of electrons from complex I to ubiquinone and transfer of ubiquinone to complex III; Can pass electrons to O2
Ubiquinone Radical
