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首页> 外文期刊>Translational research: the journal of laboratory and clinical medicine >Galactose binds to focal segmental glomerulosclerosis permeability factor and inhibits its activity.
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Galactose binds to focal segmental glomerulosclerosis permeability factor and inhibits its activity.

机译：半乳糖与局部节段性肾小球硬化通透性因子结合并抑制其活性。

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Focal segmental glomerulosclerosis (FSGS) is associated with circulating permeability activity (Palb) and recurs after transplantation in about 30% of patients. The FS permeability factor (FSPF) consists of anionic low-molecular-weight protein(s) that might be excluded by the anionic filtration barrier. We postulated that FSPF may interact with sugars of the glycocalyx, and we tested its affinity for sugars using column chromatography. FSPF showed high affinity for galactose; Palb activity was absent from unbound material and present in eluate after dialysis to remove galactose. In parallel studies, Palb activity of serum was lost after adding galactose > or = 10(-12) M. To determine whether galactose also abolishes plasma Palb activity in vivo, a patient with posttransplant FSGS was given galactose and serum samples were collected. Intravenous infusion of galactose decreased Palb from 0.88 before infusion to undetectable levels postinfusion and at 48 hours. Oral galactose diminished Palb activity; Palb reached a nadir after 2 weeks and remained low for at least 4 weeks after galactose was discontinued. We conclude that FSPF has high affinity for galactose based on chromatography. Additionally, galactose inactivates FSPF and may lead to its clearance from plasma. The interaction between FSPF and glomeruli may depend on FSPF binding to galactose, and the FSPF-galactose complex may be susceptible to uptake by galactose-binding proteins and to catabolism. We propose testing galactose as a novel nontoxic therapy for nephrotic syndrome in FSGS to determine whether galactose slows progression and whether pretransplant therapy decreases rates of recurrence and graft loss.
机译：局灶性节段性肾小球硬化症（FSGS）与循环通透性活性（Palb）相关，在约30％的患者移植后复发。 FS渗透因子（FSPF）由阴离子低分子量蛋白质组成，但阴离子过滤屏障可能会排除这些蛋白质。我们假设FSPF可能与糖萼中的糖相互作用，并使用柱色谱法测试了其对糖的亲和力。 FSPF显示出对半乳糖的高亲和力;透析除去半乳糖后，洗脱液中未存在Palb活性，且洗脱液中存在Palb活性。在平行研究中，添加半乳糖>或= 10（-12）M后，血清的Palb活性丧失。为了确定半乳糖是否也消除了体内血浆Palb活性，对移植后FSGS的患者给予了半乳糖，并收集了血清样品。静脉注射半乳糖可使Palb从注射前的0.88降至注射后和48小时无法检测到的水平。口服半乳糖会减弱Palb活性; Palb在2周后达到最低点，并且在半乳糖停用后至少保持4周低位。我们得出结论，基于色谱法，FSPF对半乳糖具有很高的亲和力。另外，半乳糖使FSPF失活，并可能导致其从血浆中清除。 FSPF和肾小球之间的相互作用可能取决于FSPF与半乳糖的结合，并且FSPF-半乳糖复合物可能易于被半乳糖结合蛋白摄取和分解代谢。我们建议测试半乳糖作为FSGS中肾病综合征的一种新型无毒疗法，以确定半乳糖是否减慢病情，以及移植前治疗是否会降低复发率和移植物丢失。

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