Elabscience

庫存單位: E-AB-53023_60μL-ES

NPHP1多株抗體，60 μL

此基因編碼具有 src 同源域 3 (SH3) 模式的蛋白質。這種蛋白質與 Crk 相關底物相互作用，似乎在控制細胞分裂以及細胞間和細胞基質黏附信號傳導中發揮作用，很可能作為位於肌動蛋白和微管結構中的多功能複合物的一部分。此基因突變會導致家族性青少年腎癆 1 型，這是一種涉及腎小管和腎小球的腎臟疾病。此基因缺陷也與 1 型 Senior-Loken 綜合徵有關，1 型也稱為伴有 Leber 黑蒙的青少年腎癆，其特徵是腎臟和眼睛疾病，以及 4 型 Joubert 綜合徵，其特徵是小腦共濟失調、眼球運動不能、精神運動遲緩和新生兒呼吸異常，有時還包括視網膜營養不良和腎臟疾病。已發現該基因有多種編碼不同異構體的轉錄變異。

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Product Specification

Basic Information

Brands:Elabscience

Model Number:

Place of Origin:中國

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Shipping:產品隨附冰袋，收到後，請立即在建議的溫度下存放。

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Calculated Molecular Weight:83千道爾頓

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Shelf Life:12個月

Storage:儲存於-20°C，有效期限為12個月。避免凍融循環。

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Calculated Molecular Weight:83千道爾頓

Dilution:免疫印跡法 1:500-1:2000

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Application:免疫組化

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Research Areas:細胞生物學；訊號傳導

Species Reactivity:人;小鼠

Reactivity:

Target Synonym:JBTS4；幼年腎癆1蛋白；腎囊素1；腎癆1（幼年）；腎癆；NPH1；NPHP1；SLSN1

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Buffer:含 0.05% NaN3 和 40% 甘油的 PBS，pH7.4

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