Biochemistry Flashcards

1
Q

_____ is deficient in mucopolysaccharidoses associated with Natowicz disease

A

Hyaluronidase
There is accumulation of Hyaluronic acid in lysosome (LSD)

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2
Q

In pentosuria, ____ is excreted in urine of patients

A

L- xylulose

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3
Q

Enzyme deficient in pentosuria

A

Xylitol reductase
In pentosuria- no symptoms other than Benedict test + . So, expectant mgmnt

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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

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