Ljungberg P
Department of Bacteriology and Immunology, University of Helsinki, Finland.
Pediatr Nephrol. 1994 Oct;8(5):531-6. doi: 10.1007/BF00858117.
The heparan sulphate proteoglycan (HSPG) of the glomerular basement membrane (GBM) is considered to be mainly responsible for the charge selectivity of the GBM; decreased HSPG results in a decreased anionic charge of the GBM with increased heparan sulphate (HS) in the urine and is believed to be responsible for the proteinuria of the congenital nephrotic syndromes (CNS). Urinary HS and chondroitin sulphate (CS) concentrations in children with CNS of the Finnish type (CNF) and the total glycosaminoglycans (GAG) in amniotic fluid of CNF pregnancies were measured by three methods: Alcian blue, Safranine O and uronic acid assays. The total urinary GAG in CNF and other nephrotic patients was comparable to controls with all three methods. Urinary CS and HS in CNF did not differ significantly from controls. Total amniotic fluid GAG was also similar in CNF and control pregnancies. These results suggest some pathogenetic mechanism other than loss of glomerular HS chains in urine for the proteinuria of CNF.
肾小球基底膜（GBM）的硫酸乙酰肝素蛋白聚糖（HSPG）被认为是GBM电荷选择性的主要决定因素；HSPG减少会导致GBM阴离子电荷减少，同时尿中硫酸乙酰肝素（HS）增加，这被认为是先天性肾病综合征（CNS）蛋白尿的原因。采用阿尔辛蓝、番红O和糖醛酸测定三种方法，对芬兰型CNS（CNF）患儿的尿HS和硫酸软骨素（CS）浓度以及CNF妊娠羊水总糖胺聚糖（GAG）进行了测定。三种方法测得的CNF及其他肾病患者的尿总GAG与对照组相当。CNF患者尿CS和HS与对照组无显著差异。CNF妊娠和对照妊娠的羊水总GAG也相似。这些结果提示，CNF蛋白尿的发病机制并非是尿中肾小球HS链丢失。
