庫存單位: E-AB-66302_200μL-ES

DLAT多株抗體,200 μL

此基因編碼多酵素丙酮酸脫氫酶複合物(PDC)的成分E2。 PDC 位於粒線體內膜,催化丙酮酸轉化為乙醯輔酶 A。該基因的蛋白質產物二氫硫辛酰胺乙醯轉移酶接受丙酮酸氧化脫羧形成的乙醯基並將其轉移到輔酶 A。二氫硫辛醯胺乙醯轉移酶是抗粒線體抗體的抗原。近 95% 的自體免疫性肝病原發性膽汁性肝硬化 (PBC) 患者體內都存在這些自體抗體。在 PBC 中,活化的 T 淋巴細胞會攻擊並破壞膽管中的上皮細胞,而這種蛋白質在膽管中異常分佈和過度表達。 PBC 最終會導致肝硬化和肝衰竭。此基因突變也是導致丙酮酸脫氫酶 E2 缺乏的原因,從而導致嬰兒期和兒童早期原發性乳酸中毒。

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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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Concentration:1毫克/毫升

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Calculated Molecular Weight:68kDa

Observed Molecular Weight:69kDa

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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:68kDa

Dilution:WB 1:500-1:2000;IHC 1:50-1:100;IF 1:50-1:200

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Features and Capabilities

Application:免疫印跡;免疫組化;免疫螢光

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Research Areas:癌症;代謝;訊號傳導

Species Reactivity:人類;老鼠;鼠

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Target Synonym:DLAT;DLTA;PDC-E2;PDCE2

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Buffer:含 0.02% 疊氮化鈉、50% 甘油的 PBS,pH7.3。

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Clonality:多克隆

Conjugation:未結合

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