TCA cycle Flashcards
location of TCA cycle?
Mitochondira
Most cells contain how many mitochondria?
500-2,000
Primary role of Mitochondria is?
ATP production via aerobic respiration
What is a common product in the catabolism of carbohydrates (glycolysis), amino acids and fatty acids (B-oxidation)?
Acetyl- CoA
Catabolism?
Breakdown
Anabolism?
Synthesis of molecules
How is TCA a amphibolic pathway?
because it involves both catabolism and anabolism
Acetyl Coa has how many carbons?
2 carbons
Oxaloacetate has how many carbons?
4 carbons
Acetyl- CoA to Citrate. What is released?
CoA is released
Citrate has 6 carbons.
Isocitrate to alpha ketoglutarate
NADH produced
CO2 released
Alpha-keto = 5 carbons
Alpha- ketoglutarate to succinyl-CoA ?
CoA added
CO2 released
NADH produced
Succinyl-CoA = 4 carbons
Succinyl-CoA to Succinate
GTP and CoA released.
Succinate to Fumurate
FADH2 released
Fumurate to malate
H20 added
Malate to Oxaloacetate
NADH produced
energy rich components are added into the TCA cycle where?
Acetyl-CoA is added in the beginning of TCA and succinly-coA.
Oxidative Phosphorylation happens in the?
Inner mitochondrial matrix
Metabolic Fates of Pyruvate.
Pyruvate is reduced to what and with what enzyme?
Reduced to Lactate
via lactate dehydrogenase
Metabolic Fates of Pyruvate
When Pyruvate is oxidative decarboxylation it becomes?
With what enzyme?
Acetyl-CoA
3 carbon to 2 carbon via pyruvate dehydrogenase complex.
Metabolic Fates of Pyruvate.
When Pyruvate is carboxylized it becomes?
with what enzyme?
Pyruvate carboxylase
and it becomes oxalacetate.
Pyruvate is transamination
what does it become via what enzyme?
becomes alanine
via alanine aminotransferase
A porin in the mitochondria?
a special carrier for pyruvate involving a symport that utilizes the H+ gradient from outside to inside the mitochondrion.
High selective transporters are where in the mitochondria?
inner!
Pyruvate dehydrogenase complex (PDC) is a complex
of what three enzyme activities that transform pyruvate into acetyl-CoA by a process called pyruvate decarboxylation?
- pyruvate dehydrogenase
- dihydrolipoyl tranacetylase with its lipoic acid prosthetic group
- dihydrolipyl dehydrogenase
Pyruvate dehydrogenase is inhibited when one or more of the three following ratios are increased:
ATP/ADP
NADH/NAD+
Acetyl-CoA/CoA
Acetyle-Coa , NADH and Pyruvate are ?
allosteric inhibitors
- they upregulate PDH kinase which renders the PDH complex inactive
- Acetyl-Coa can also Allosterically directly inhibit the PDH complex
Phopshorlaytion of PDH kinase
Inactive PDH Complex
dephosphorylation of PDH kinase
Active PDH complex
PDH phosphatase upregulation
active PDH complex
Insulin and cofactors
upregulate PDH phosphatase
which
activates PDH complex
What makes Acetyl- CoA?
Fatty Acids
Pyruvate
Amino Acids
Acetyl-Coa can produce?
Ketone bodies
Tricarboxylic acid
sterols
fatty acids
Acetyl- Coa to Citrate?
enzyme
citrate synthesis
isocitrate to alpha keto
RLE is : isocitrate dehydrogenase
alpha keto to succuniyl coa
alpha-keto dehydrogenase
succiynl coa to succinate
succinyl coa synthetase
succinate to fummurate
succinate dehydrogenase
malate to oxaloacetate
malate dehydrogenase
Energy producing steps and also sites of regulation
Citrate synthase
Isoitrate Dehydrogenase
Alpha keto dehydrogenase
TCA cycle inhibition for Citrate Synthase
Inhibition: ATP
NADH
Citrate
Succinyl-CoA
Activation: ADP
TCA cycle activation
by ADP and CA
TCA cycle needs both?
acetyl-coa and oxolacetate
Pyruvate to Oxoalacetate needs what cofactor to fxn with enzyme?
biotin with pyruvate decoarboxylase
TCA releases both Carbons from Acetyl-Coa as?
Co2
TCA cycle produces?
3 NADH
1 FADH2
1 GTP
Isocitrate dehydrogenase is what in TCA?
its the rate limiting step and most important and controlled by ATP and NADH
TCA cycle serves as a
metabolic traffic circle that receives carbon skeletons from amino acids and fatty acids and donates carbon skeletons to amino acids and porphyrins
Genetic mutations affecting the TCA cycle and accumulation of TCA cycle intermediates (???) or TCA cycle substrates (???) appear to be particularly toxic to neurons and brain development (encephalopathies).
Genetic mutations affecting the TCA cycle and accumulation of TCA cycle intermediates (succinate, fumarate) or TCA cycle substrates (pyruvate) appear to be particularly toxic to neurons and brain development (encephalopathies).
Pyruvate dehydrogenase deficiency, leading to what?
Children with this disease frequently exhibit severe (blank) defects; resulting in death in many cases.
Dietary management may include?
leading to chronic lactic acidosis
severe neurological defects
ketogenic diet
Fumarase deficiency?
Urine contains?
Rare autosomal recessive disease with severe neurological impairment.
Urine contains:
abnormal amounts of fumarate
and elevated levels of succinate
and other TCA cycle intermediates.
TCA cycle dysfunctions can lead to
encephalopathies and certain types of cancer.
TCA cycle inhibition for Isocitrate Dehydrogenase
ATP
TCA cycle inhibition for Alpha keto dehydrogenase
NADH, Succinyl-Coa
TCA cycle Activation for alpha keto dehydrogenase
Ca 2+
TCA cycle Activation for Isocitrate dehydrogenase
Ca 2+, ADP
TCA cycle activation for citrate synthase
ADP