Special Topics

Question 1

Long, unbranched heteropolysaccharide chains generally composed of a repeating disaccharide unit of amino sugar and acidic sugar

Answer 1

Glycosaminoglycans

Question 2

Only GAG that is not covalently bonded to proteins to form proteoglycan

Answer 2

Hyaluronic acid

Question 3

GAGs are covalently bonded to protein forming proteoglycans via

Answer 3

Trihexoside serine O-glycosidic bond

Question 4

Important role in permitting cell migration during morphogenesis and wound repair and contributea to compressibility of cartilage

Answer 4

Hyaluronic acid

Question 5

GAG that is located at sites of calcification in endochondral bone and are also found in cartilage

Answer 5

Chondroitin sulfate

Question 6

2 GAGs that play a critical role in corneal transparency

Answer 6

Keratan sulfate I and dermatan sulfate

Question 7

GAG which is an important anticoagulant

Answer 7

Heparin

Question 8

GAG which is a component of plasma membranes where they may act as receptors and participate in cell adhesion and cell-cell interactions

Answer 8

Heparan sulfate

Question 9

GAG that determine the charge selectiveness of renal glomerulus

Answer 9

Heparan sulfate

Question 10

Accumulation of GAGs in the lysosome due to deficiency in hydrolases

Answer 10

Mucopolysaccharidoses

Question 11

All mucopolysaccharidoses are autosomal recessive except for

Answer 11

Hunter’s syndrome (X-linked)

Question 12

All mucopolysaccharidoses causes mental retardation except for

Answer 12

Morquio’s syndrome

Question 13

Type I mucopolysaccharidoses

Answer 13

Hurler’s syndrome

Schele’s syndrome

Question 14

Type II mucopolysaccharidoses

Answer 14

Hunter’s syndrome

Question 15

Type III mucopolysaccharidoses

Answer 15

Sanfilippo syndrome

Question 16

Type IV mucopolysaccharidoses

Answer 16

Morquio’s syndrome

Question 17

Type VII mucopolysaccharidoses

Answer 17

Sly syndrome

Question 18

Deficiency in alpha-L-iduronidase

Answer 18

Type I mucopolysaccharidoses

Hurler - with corneal clouding and cardiomyopathy
Schele’s

Question 19

Deficiency in iduronate sulfatase

Answer 19

Type II mucopolysaccharidoses or Hunter’s syndrome

No corneal clouding

With cardiomyopathy and mental retardation

Question 20

Deficiency of heparan sulfamidase

Answer 20

Type IIIA mucopolysaccharidoses

All type III are Sanfilippo syndromes

Question 21

Deficiency in N-acetylglucosaminidase

Answer 21

Type IIIB mucopolysaccharidoses

All type III are Sanfilippo syndromes

Question 22

Deficiency in N-acetylglucosamine sulfatase

Answer 22

Type IIIC mucopolysaccharidoses

All type III are Sanfilippo syndromes

Question 23

Deficiency in N-glucosamine

Answer 23

Type IIID mucopolysaccharidoses

All type III are Sanfilippo syndromes

Question 24

Deficiency in galactose-6-sulfatase

Answer 24

Type IV mucopolysaccharidoses or Morquio’s syndrome

Question 25

Deficiency in beta glucuronidase

Answer 25

Type VII mucopolysaccharidoses or sly syndrome

Corneal clouding
Hepatimegaly
Skeletal dysplasia and short stature
Mental retardation

Question 26

Deficient phosphorylation of mannose residues in N-linked glycoprotein pre-enzymes

Answer 26

I cell disease