MUCOPOLYSACCHARIDE DISORDERS

Question 1

MUCOPOLYSACCHARIDOSES

Answer 1

HURLER, HUNTER, SAN FILIPPO

Question 2

Mucopolysaccharidoses is an impaired metabolism of

Answer 2

MPS or GLYCOSAMINOGLYCANS

Question 3

Glycosaminoglycans is a combination of

Answer 3

PROTEIN and POLYSACCHARIDE

Question 4

Where are glycosaminoglycans located

Answer 4

CONNECTIVE TISSUE

Question 5

MPS frequently found in the urine

Answer 5

Dermatan sulfate
Keratan sulfate
Heparan sulfate

Question 6

ABNORMALITIES

1. Hurler
2. Hunter
3. San Filippo

Answer 6

1. Skeletal abn and mental retardation
   MPS accumulate in the cornea
2. Skeletal abn and mental retardation
3. Mental retardation

Question 7

SCREENING TESTS FOR MPS (+ result)

Answer 7

1. ACID ALBUMIN : white turbidity
2. CETYLTRIMETHYLAMMONIUMBROMIDE (CTAB) test: white turbidity
3. MPS PAPER TEST: Blue