庫存單位: PKSH033249_10μg-ES

重組人類 α-半乳糖苷酶 A/GLA 蛋白 (His 標籤),10 μg

α-半乳糖苷酶 A 是一種同型二聚體糖蛋白,屬於醣基水解酶 27 家族。它是一種溶酶體酶,用於確診為法布瑞氏症的患者的長期酶替代療法。 α-半乳糖苷酶A可以水解醣脂和醣蛋白中的末端α-半乳糖基部分,並催化蜜二糖水解為半乳糖和葡萄糖。缺陷的 α-半乳糖苷酶 A 是法布瑞氏症 (FD) 的病因,這是一種罕見的 X 連鎖鞘脂類沉積症,醣脂會在許多組織中積聚。此疾病是由於先天性的糖鞘脂分解代謝錯誤所引起的。 FD 患者表現出全身血漿和細胞溶酶體中糖鞘脂 (Gb3) 和相關糖鞘脂的系統性累積。患者可出現眼部沉積、發燒和四肢灼熱等症狀。死亡是由於腎衰竭、高血壓或其他血管疾病的心臟或腦部併發症造成的。

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Product Specification

Basic Information

Brands:Elabscience

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Place of Origin:中國

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Reconstitution:不適用

Shipping:本產品以液體形式提供。它是在冷凍溫度下用藍冰/凝膠包運輸的。收到後,立即存放在<-20°C的環境中。

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Calculated Molecular Weight:46.4 kDa

Observed Molecular Weight:50-60kDa

Purity: 以還原 SDS-PAGE 測定,結果 > 95%。

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Storage:儲存溫度<-20°C,可穩定保存6個月。請盡量減少凍融循環。

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Calculated Molecular Weight:46.4 kDa

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Endotoxin: 經 LAL 方法測定,每 μg 蛋白質含量 < 1.0 EU。

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Research Areas:心血管

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Bio-activity:未驗證活動

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Formulation:以 20mM Tris-HCl、150mM NaCl、pH 8.0 的 0.2 μm 過濾溶液形式提供。

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