BIOMOLECULES AND ENZYMES

BIOLOGY

CLASSIFICATION OF CARBOHYDRATES

Question [CLICK ON ANY CHOICE TO KNOW THE RIGHT ANSWER]
Deficiencies in galactosamine 6-sulfatase and B-galactosidase that degrade keratan sulfate result in ____
A
Hurler syndrome
B
Sanfilippo syndrome
C
Maroteaux-Lamy syndrome
D
Morquio syndrome
Explanation: 

Detailed explanation-1: -Morquio A syndrome is an autosomal recessive disorder, one of 50 lysosomal storage diseases (LSDs), and is caused by the deficiency of N-acetylgalactosamine-6-sulfate sulfatase (GALNS). Deficiency of this enzyme causes specific glycosaminoglycan (GAG) accumulation: keratan sulfate (KS) and chondroitin-6-sulfate (C6S).

Detailed explanation-2: -Causes of Morquio Syndrome A mutation is the GALNS gene, which encodes for N-acetyl galactosamine-6-sulfatase, causes Morquio, type A. Type B is caused by mutations of the GLB1 gene, which encodes for -galactosidase. Both enzymes, however, are responsible for keratan sulfate degradation.

Detailed explanation-3: -N-acetylgalactosamine-6-sulfatase is an enzyme that, in humans, is encoded by the GALNS gene. Chr. Chr. This gene encodes N-acetylgalactosamine-6-sulfatase, which is a lysosomal exohydrolase required for the degradation of the glycosaminoglycans keratan sulfate and chondroitin 6-sulfate.

Detailed explanation-4: -Diagnosis of Morquio syndrome starts with a thorough medical history and physical exam. Your child’s doctor might order: genetic testing. x-ray images to produce images of your child’s bones.

There is 1 question to complete.