All of the following are Glycoproteinoses, EXCEPT
Ans: b) Sanfilippo A syndrome Sanfilippo A syndrome is a mucopolysaccharidosis, not Glycoproteinoses. Lysosomal hydrolases like a-neuraminidase, b-galactosidase, b-hexosaminidase are involved in the degradation of oligosaccharide chains during glycoprotein turnover. Enzyme defects lead to abnormal degradation of glycoproteins and accumulation of paially degraded glycoproteins in tissues, known as Glycoproteinoses. Glycoproteinoses are classified into lysosomal storage disorders. All Glycoproteinoses are associated with mental retardation. autosomal recessive mode. Glycoproteinoses Defective enzyme Fucosidosis a-Fucosidase a-Mannosidosis a-Mannosidase b-Mannosidosis b-Mannosidase Aspaylglucosaminuria Aspaylglucosaminidase Sialidosis Neuraminidase
Fucosidosis
Sanfilippo A syndrome
Sialidosis
a-mannosidosis
Classification of carbohydrate