Please enable JavaScript.
Coggle requires JavaScript to display documents.
Pyruvate Dehydrogenase Complex (PDH) Pyruvate --> Acetyl-CoA (3…
Pyruvate Dehydrogenase Complex (PDH)
Pyruvate --> Acetyl-CoA
(3 carbon) --> (2 carbon)
1 NADH/pyruvate
Pyruvate
end product of
aerobic glycolysis
major source of
Acetyl-CoA
(converted via PDH)
Transported into mitochondria
"not part of TCA cycle per se"
COENZYMES
bind to enzyme
Cofactors
E1
TPP: thiamine (
Vitamin B1
) pyrophosphate-E1
E2
Lipoic acid
Coenzyme A (CoA)
E3
NAD
FAD
REGULATION
E1
PDH phosphate
activates
E1
PDH kinase
inhibits
E1
Pyruvate inhibits PDH kinase!
Skeletal Muscle Cells:
Release of Ca2+ during contraction stimulated PDH & energy production by activating PDH Phosphatase
DEFICIENCY
E1 defiency
Lactic acidosis
(pyruvate shunted to lactic acid via LDH)
Brain sensitive to lactic acidosis
E1=
X-linked dominant gene
NO proven treatment for PDH deficiency
Arsenic Poisoning
Arsenite
PDH, a-ketoglutarate dehydrogenase, and more
forms stable complex with
thio group
of lipoid acid
inhibits enzymes needing
lipoid acid
Binds to lipoic acid, causing
inactivation
of the
E2
enzyme -> lactic acidosis
Affects brain -> neurological disturbance & death
a-keto acid dehydrogenase family
PDH: E1, E2, E3 Enzymes
E1
: Pyruvate dehydrogenase
E2
: Dihydrolipoyl transacetylase
E3
Dihydrolipoyl dehydrogenase