Summary of Pathways

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74 SECTION II BIOCHEMISTRY BIOCHEM ISTRY-METABOLISM

Summary of pathways

O Galactokinase (mild galactosemial Galactose metabolism


E) Galactose-1-phosphate Ga lactose Requires biotin cofactor
uridyltransterase
(severe galactosemial 10 Glycolysis
I Requires thiamine cofactor ITPPJ
( ) Irreversible. important point of regulation
E) Hexokinase/glucokinase Glycogen Glulc!ose
0 Glucose-6-phosphatase
Ivon Gierke disease)
t "-- a o e HMPshunt
UDP-glucose +-- Glucose-I-phosphate .=:; 6-phosphogluconolactooe)
0 Glucose-6-phosphate
dehydrogenase Glycogenesis / glycogenolysis l! <:)
0 fructose-6-phosphate - Ribulose-5-pllosphate
Of!O
Transketolase
f) Phosphotructokinase-1 Gluconeogenesis T

() Fructose-1.6-bisphosphatase Fructose-t6i l ; ospl1ate


e Fructokinase (essential fructosuna)
e Aldolase B(fructose intolerance) Giyceraldehyde-3-P .=::; DHAP +--Fructose-I-phosphate +-- Fructose

..
6) Aldolase B(liver). A(muscle)
® Triose phosphate isomerase
e Pyruvate kinase 3-phosphoglycerate Glycerol lipid metabolism
4t) Pyruvale dehydrogenase
G) Pyruvate cart>oxylase u
2-phosphoglycerate
r
Triglycerides
PEP carboxykinase
u r
f
'1) Citrate synthase
Pllosphoenolfeate !PEPI Fatty acids
Cll lsocitrate dehydrogenase
Ii) cx-ketoglutarate dehydrogenase Alanine :;::::::::'.: Pyruvate .!::::; Lactate rC Cholesterol
Carbamoyl phosphate synthetase I T / ionyl- oA l
8
ED transcarbamylase e Acetyl-CoA E!J Mevalonate
e Propionyl-CoA carboxylase \ Acetoacetyl-CoA--. HMG-CoA
E!J HMG-CoA reductase Acetoacetate
1
1
Asparlate Oxaloacetate "-
NH3+C02
fl) Citrulhn
e--l'\. ( "\
lsocitrate Jl-hydroxybutyrate .

Cart>amoyl phosphate --(m Arg1ninosuccinate Malate TCA cycle )e Ketogenesis

Ornithine \ Odd-chain fatty acids.


Urea cycle cx-ketoglutarate branched-chain amino acids.
Fumarate Lk methionine andthreonine
g1nine "- _/11:) e. !
U Suwnale Suwnyl-CoA +-- Methylmalonyl-CoA +-- Prop1onyl-CoA
re - B12 B

HzO Protein metabolism

ATP production Aerobic metabolism of one glucose molecule Arsenic causes glycolysis to produce zero net
produces 32 net ATP via ma late-aspartate ATP.
shuttle (heart and liver}, 30 net ATP via
glycerol-3-phosphate shuttle (muscle).
Anaerobic glycolysis produces only 2 net ATP
per glucose molecule.
ATP hydrolysis can be coupled to energetically
unfavorable reactions.

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