Uronic Acid Pathway Flashcards
A pathway for conversion of glucose to glucoronic acid, ascorbic acid and pentoses
Alternative oxidative pathway for glucose but it does not lead to generation of ATP
Uronic Acid Pathway
Serves as precursor of ascorbic acid in other animals
Glucoronic acid
Humans lack this enzyme important for conversion of L-gulonolactone to L-ascorbic acid
L-gulonolactone oxidase
First step:
Glucose -> Glucose 6 Phosphate by enzyme Glucokinase
Step 2
Glucose 6-phosphate ->
by enzyme
Glucose-1-Phosphate
Phosphoglucomutase
Step 3
Glucose-1-phosphate ->
by enzyme
Used up
UDP glucose by enzyme UDP glucose prophosphorylase
UTP to become PPi
Step 4
UDP Glucose ->
by enzyme
Gives off
UDP glucoronate/
UDP glucoronic acid by enzyme UDP glucose dehydrogenase
2 NADH and H20
Step 5
UDP glucoronic acid ->
releases
D glucoronic acid by enzyme UDP glucoronidase
1 UDP
Step 6
D Glucoronic acid ->
Uses up
L gulonate by enzyme glucoronate reductase
NADPH -> NADP
In lower animals, L gulonate will be converted into
L gulonolactone by enzyme Aldonolactonase
L gulonolactone will be converted into 2 ketogulonolactone by enzyme Gulonolactone oxidase that is missing in humans
2 ketogulonolactone is converted into L ascorbic acid
In humans, L gulonic acid is converted into
3 keto L gulonate by enzyme L gulonate oxidase with conversion of NADP - NADPH
L xylulose will be converted into xylitol by xylulose reductase using ip NADH
D xylulose will be converted to D Xylulose 5 Phosphate by enzyme xylulose kinase
The metabolite from D glucoronic acid that enters PPP
D xylulose 5 phosphate
Inborn error of metabolism
One of the members of Garrod’s tetrad
Essential pentosuria
Absence of these enzymes lead to pentosuria
Xylitol dehydrogenase
Xylulose reductase
L xylulose in urine on pentosuria gives positive
Benedict’s test