免疫刺激诱导的黏多糖贮积症IIIA患者硫酸乙酰肝素动员

Mobilization of heparan sulfate induced by immunostimulation in a patient with mucopolysaccharidosis IIIA.

作者信息

Zerial M, Florio C, Dolzani L, Moro L, Romeo D

出版信息

Biochem Med. 1984 Apr;31(2):228-35. doi: 10.1016/0006-2944(84)90027-9.

Abstract

Unspecific immunostimulation by bacterial vaccines of a patient with mucopolysaccharidosis IIIA (Sanfilippo A) syndrome induces a marked increase in the urinary excretion of heparan sulfate and of uronate-containing oligosaccharides. This event is presumably linked to an increased vascular permeability and exocytosis of storage material, elicited by mediators of inflammation, as well as to enhanced degradation of stored polymers in activated macrophages and surrounding tissue.

摘要

粘多糖贮积症IIIA型（Sanfilippo A综合征）患者接种细菌疫苗后产生的非特异性免疫刺激会导致硫酸乙酰肝素和含糖醛酸寡糖的尿排泄量显著增加。这一现象可能与炎症介质引发的血管通透性增加和储存物质的胞吐作用有关，也与活化巨噬细胞和周围组织中储存聚合物的降解增强有关。