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HMP Shunt Pathway
UC MED 2015
30
Medical
Graduate
10/24/2011

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Cards

Term
What do the oxidative pathways form in the HMP Shunt pathway?
Definition
NADPH
Term
What do the non-oxidative pathways form in the HMP Shunt pathway?
Definition
The non-oxidative reactions allow five carbon sugars to be converted to six carbon sugars, using reversible reactions
Term
What enzyme transfers two carbons between sugars? And where is it located?
Definition
Tranksketolase-found in the cytoplasm
Term
What cofactor is required by Transketolase?
Definition
thiamine pyrophosphate- thus this can be utilized as a measurement for thiamine deficiency
Term
What enzyme transfers 3 carbons between sugars? And where is it located?
Definition
Transaldolase-located in the cytoplasm
Term
Transaldolase requires what residue at its active site?
Definition
Lysine
Term
What vitamin deficiency causes these two sets of symptoms...

1)fluid retention-affecting the cardiovascular system
2) pain, paralysis- affecting the nervous system
Definition
Thiamine deficiency- causes Beriberi
1) wet beriberi
2) dry beriberi
Term
What disease caused from a vitamin deficiency of thiamine? Cause these symptoms...
-Primarily seen in alcoholics
-Alcohol impairs thiamine absorption
-Neuronal dysfunction (lack of energy due to lack of pyruvate dehydrogenase activity)
Definition
Wernicke-Korsakoff syndrome
Term
How can you test for thiamine deficiencies?
Definition
measuring transketolase activity in red blood cells
Term
What enzyme does this reaction...
Glucose-6-Phosphate +NADP+ -->
6-Phosphogluconolactone + NADPH
Definition
Glucose-6-Phosphate Dehydrogenase
Term
What enzyme does this reaction...
6-Phospho-gluconate + NADP+ -->
Ribulose-5-Phosphate + CO2 + NADPH
Definition
6-Phospho-gluconate Dehydrogenase (it is an oxidation rxn followed by a decarboxylation)
Term
Ru5P
Definition
Ribulose-5-Phosphate
Term
Is Ru5P a substrate for TK or TA?
Definition
No
Term
What enzyme converts Ru5P to X5P?
Definition
C3-Epimerase
Term
What enzyme converts Ru5P to R5P?
Definition
Isomerase
Term
Other than Ru5P to X5P, what other rxn has a epimerization?
Definition
Galactose metabolism
Term
The HMP shunt pathway and glycolysis intersect at what compounds?
Definition
F6P and G3P
Term
What activates G6PDH?
Definition
NADP+
Term
If the cell requires more R5P than NADPH, what happens?
Definition
Use TK and TA to generate R5P from F6P and G3P

Bypass the Oxidative steps
Overall rxn is...
5 G6P + ATP --> 6 R5P + ADP + H+
Term
What happens if the cell needs equal amounts of NADPH and R5P?
Definition
Use only the Oxidative pathway: requiring the enzymes G6PDH and 6-PGDH plus isomerase

Overall rxn:
G6P + 2NADP+ + H2O --> CO2 +R5P + 2NADPH + 2H+
Term
If the cell requires NADPH, but not R5P, what happens?
Definition
In this case, go through the oxidative rxns to form NADPH and Ru5P, then use the non-oxidative rxns and gluconeogenesis to convert Ru5P to glucose.

The overall rxn is:
G6P +12NADP+ + 7H2O --> 6CO2 + 12NADPH + 12H+ + Pi
Term
What happens if the cell requires NADPH and carbons for biosynthesis?
Definition
Take G6P through the oxidative rxns to generate NADPH and Ru5P. Then take Ru5P and produce pyruvate by combing TK, TA and glycolysis

Overall rxn is:
3 G6P + 6NADP+ + 5 NAD+ + 5 Pi + 8 ADP -->
5 pyruvate +3 CO2 + 6NADPH +5NADH + 8 ATP +2 H2O + 8 H+
Term
What pathway requires NADPH?
Definition
fatty acid synthesis
Term
What enzyme found in the mitochondria will do this... convert NADH + NADP+ into NAD+ and NADPH in a reversible reaction?
Definition
transhydrogenase
Term
What cells are primarily affected by a G6PDH deficiency?
Definition
red blood cells-since they lack mitochondria which has transhydrogenase
Term
What small peptide found in the red blood cell protects itself from oxidative damage?
Definition
glutathione
Term
Glutathione is normally reduced, after being oxidized by an oxidizing agent, it needs to be reduced by what?
Definition
NADPH and the enzyme glutathione reductase
Term
If there is a G6PDH deficiency then...
Definition
the red blood cell membrane is vulnerable to oxidative damage once all the glutathione in the membrane had been oxidized
Term
G6DPH deficiency is what type of genetic disorder and found mostly in what ethnic group?
Definition
It is X-linked and commonly found in people of Middle Eastern descent
Term
When is a G6PDH deficiency seen?
Definition
Only under oxidative stress- Not too many oxidizers in our body. Pt should never take anti-malarial drugs since they are oxidizing agents and the mere presence of these drugs will cause hemolytic anemia. (This disease naturally protects a person from malaria, so similar to sickle-cell anemia).
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