Question

An infant presented with multiple skeletal abnormalities, with the most prominent ones being a short trunk and short limbs. Urine was free of partially degraded oligosaccharides or oligosaccharides of protcoglycans. The blood sulfate concentration and extracellular acid hydrolase activity were normal. Sulfation is an important component of the synthesis of A. most proteoglycans. B. hyaluronate. C. most glycoproteins. D. conjugated bilirubin. E. all of the above.

Short answer

Answer: A) most proteoglycans

Step-by-step solution

Analyze the presence of sulfate

In order to identify which molecules' synthesis is related to sulfation, we need to first consider the given information: blood sulfate concentration and extracellular acid hydrolase activity were normal, which means the sulfation process might not be affected in this case.

Analyze the role of sulfation

Sulfation is a process that adds a sulfate group to a molecule. Now let's analyze the role of sulfation in synthesizing the given molecules: - A) Most proteoglycans: Sulfation is important for the synthesis of proteoglycans, as they have sulfated glycosaminoglycan (GAG) chains attached to a core protein, which play a crucial role in their structure and function. - B) Hyaluronate: Hyaluronate is a glycosaminoglycan but does not contain sulfate, so sulfation is not involved in its synthesis. - C) Most glycoproteins: Sulfation does occur in some glycoproteins, but not most of them, so this statement is not accurate. - D) Conjugated bilirubin: Sulfation is not directly related to conjugated bilirubin synthesis. - E) All of the above: This is not an accurate statement as hyaluronate, most glycoproteins, and conjugated bilirubin are not directly related to sulfation.

Determine the correct answer

Based on our analysis, we can see that sulfation is an important component in the synthesis of most proteoglycans (option A). The other options are not directly related to sulfation. Thus, the correct answer is A) most proteoglycans.