HMP shunt and other sugars Flashcards
1
Q
What things is NADPH used for
A
- Synthesis of fatty acids and cholesterol
- Generation of oxygen free radicals
- Protection of RBCs from oxygen free radicals
- Cytochrome P450 enzymes
2
Q
In patients with G6PD deficiency, ingestion of what substances can induce oxidative damage to RBCs?
A
- “Spleen Purges Nasty Inclusions From Damaged Cells”
- Sulfonamide
- Primaquine
- Nitrofurantoin
- Isoniazid
- Fava beans
- Dapsone
- Chloroquine
3
Q
Describe Essential Fructosuria
A
- Deficiency in FructoKINase (KIND disease)
- Fructose in urine but otherwise benign
4
Q
Describe Fructose intolerance
A
- Aldolase B deficiency (B for Bad)
- Fructose-1-P buildup
- Using alot of phosphates
- Ends up inhibiting glycogenolysis and gluconeogenesis
- Wont be able to correct fasting hypoglycemia
- Hypoglycemina
- Vomiting
- Hepatosplenomegaly
- Jaundice
5
Q
Treatment of Fructose intolerance
A
-decrease intake of fructose and sucrose
6
Q
Describe Galactokinase deficiency
A
- increase in Galactitol
- Cataracts in infants
7
Q
Describe Classic Galactosemia
A
- Absence of Gal-1-P uridyltransferase
- Same symptoms as Galactokinase deficiency but worse
- Hepatosplenomegaly
- Jaundice
- Failure to Thrive
- Intellectual Disability
8
Q
Treatment for Classic Galactosemia
A
- Exclude Galactose from diet
- Exclude Lactose also: it is a dimer of Glucose and galactose
9
Q
Rate limiting step of pentose phosphate pathway
A
G6PD
10
Q
What tissues of the body use the pentose phosphate pathway
A
- RBCs
- Liver
- Adrenal Cortex
- Mammary glands
