Metabolism I Flashcards
beta ox location
mitochondria
TCA cycle location
mitochondria
ox phos location
mito
HMP shunt location
cytoplasm
glycolysis location
cytoplasm
kinase
add P using ATP
phosphorylase
add P without using ATP
phosphatase
remove P
dehydrogenase
catalyze redox rxn
hydroxylase
add OH group
carboxylase
transfer CO2 group
require biotin
mutase
relocate functional group
glycolysis rate limiting step
PFK-1
gluconeogenesis rate limiting step
fructose 1,6 bisphosphatase
TCA cycle rate limiting step
isocitrate DH
glycogenolysis rate limiting step
glycogen phosphorylase
HMP shunt rate limiting step
G6PD
de novo pyrimidine synthesis rate limiting step
CPS II
de novo purine synthesis rate limiting step
PRPP amidotransferase
urea cycle rate limiting step
CPS I
fatty acid synthesis rate limiting step
acetyl CoA carboxylase
ACC
fatty oxidation rate limiting step
carnitine acyltransferase I
ketogenesis rate limiting step
HMG CoA synthase
choletserol synthesis rate limiting step
HMG-CoA reductase
fructose 2,6 bisphoshate regulation
stimulate PFK-1 - glycolysis
inhibit fructose 1,6 - bisphosphatase - gluconeogenesis
regulation isocitrate dehydrogenase
rate limiting step - TCA cycle
inhibitor - ATP and NADH
stimulation - ADP
malate aspartate shuttle
heart and liver
produce 32 ATP from glucose
glycerol-3 phosphate shuttle
muscle
produce 30 ATP from glucose
arsenic
cause glycolysis produce 0 ATP
vit B3
source of NAD+
vit B2
source of FAD+
NADPH production
HMP shunt
hexokinase
most tissue - not liver and beta cells
low Km
low Vmax
feedback inhibition - glucose 6 P
mutation with maturity onset diabetes of young
glucokinase
glucokinase
liver and pancreatic beta cells
high Km
high Vmax
induced by insulin
hexokinase vs. glucokinase
low glucose - hexokinase sequester in tissue
high glucose - glucokinase store in liver
fructose 2,6 biphosphate regulation
FBP-ase 2 and PFK-2 - part of same enzyme
- PFK-2 produces fructose 2,6 biphosphate (stimulate PFK-1)
- FBPase-2 produces fructose 6-P (gluconeogenesis)
fasting state - glucagon - enzyme P - FBP-ase 2 activity - more gluconeogenesis
fed state - insulin - enzyme de-P - PFK-2 activity - more glycolysis
pyruvate dehydrogenase
links glycolysis and TCA cycle
-active in fed state
pyruvate > acetyl CoA
requires cofactors - TLCFN
TLCFN
cofactors
thiamine -B1 lipoic acid CoA - B5 FAD - riboflavin - B2 NAD - niacin - B3
necessary for
- pyruvate DH
- a-KG DH - TCA cycle
increased serum alanin, neuro deficit, lactic acidosis
deficient pyruvate DH complex
build up of pyruvate - shunted to lactate and alanine production
tx pyruvate DH deficiency
high fat diet
high lysine and leucine - ketogenic AAs
low glucose
pyruvate has what possible pathways
alanine
OAA
acetyl CoA
lactate
irreversible enzymes of TCA cycle
citrate synthase
isocitrate DH - rate limiting
a-KG DH
products of TCA cycle
3 NADH
1 FADH2
2 CO2
1GTP
occurs 2x - 2 acetyl CoA formed per glucose
TCA cycle location
mitochondria
pyruvate carboxylase
pyruvate > OAA
pyruvate dehydrogenase
pyruvate > acetyl CoA
alanine aminotransferase
pyruvate > alanine
lactic acid dehydrogenase
pyruvate > lactate