Biochemistry Flashcards
1
Q
_____ is deficient in mucopolysaccharidoses associated with Natowicz disease
A
Hyaluronidase
There is accumulation of Hyaluronic acid in lysosome (LSD)
2
Q
In pentosuria, ____ is excreted in urine of patients
A
L- xylulose
3
Q
Enzyme deficient in pentosuria
A
Xylitol reductase
In pentosuria- no symptoms other than Benedict test + . So, expectant mgmnt
4
Q
H/o strenuous exercise, skipped meal, consumed alcohol afterwards. Why would hypoglycemia develop?
A
Alcohol increases NADH levels–> it will increase conversion of OAA to malate (which requires NADH). OAA (substrate for gluconeogenesis) decreases so gluconeogenesis stops