產品名稱：Anti-PDCE2抗體,丙酮酸脫氫酶E2抗體*

產品編號：BYK-19695R
英文名稱：Anti-PDCE2
中文名稱：丙酮酸脫氫酶E2抗體
英文別名：Pyruvate Dehydrogenase E2;

70 kDa mitochondrial autoantigen of primary biliary cirrhosis;

anti DLAT;

Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex;

Dihydrolipoamide;

Dihydrolipoamide S Acetyltransferase;

Dihydrolipoamide S-acetyltransferase (E2 component of pyruvate dehydrogenase complex);

Dihydrolipoamide S-Acetyltransferase;

Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex;

dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial;

DLAT;

DLAT; ;

DLTA;

E2;

E2 component of pyruvate dehydrogenase complex;

EC 2.3.1.12;

M2 antigen complex 70 kDa subunit;

M2 Antigen Complex 70kD Subunit;

mitochondrial;

ODP2_HUMAN;

PBC;

PDC E2;

PDC-E2;

PDCE2;

Pyruvate dehydrogenase complex component E2;

Pyruvate dehydrogenase complex E2 subunit;

S acetyltransferase component of pyruvate dehydrogenase complex.
英文介紹：This gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC resides in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, accepts acetyl groups formed by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC enventually leads to cirrhosis and liver failure. Mutations in this gene are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lactic acidosis in infancy and early childhood.

產品規格：0.1ml/0.2ml

注意事項:This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

此抗體本公司有單抗和多抗，可用于免疫組化（IHC），免疫印跡（WB），酶聯免疫(ELISA)，IF、IP、流式細胞術等實驗（可來電索取說明）
此產品本公司有標記的一抗出售，相關標記有：Alexa Fluor 350 標記、Alexa Fluor 488 標記、Alexa Fluor 555 標記、Alexa Fluor 647 標記、AP標記、APC標記、Biotin標記、Cy3標記、Cy5標記、Cy5.5標記、Cy7標記、FITC標記、Gold標記、HRP標記、PE標記、PE-Cy3標記、PE-CY5標記、PE-CY5.5標記、PE-CY7標記、RBITC標記

保存條件：Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

Anti-PDCE2抗體,丙酮酸脫氫酶E2抗體*免疫組化染色：
SP法
（1） 脫蠟、水化 （2）PBS洗2-3次各5分鐘 （3）3%H2O2（80%甲醇）滴加在
TMA上，室溫靜置10分鐘 （4）PBS洗2-3次各5分鐘 （5）抗原修復
（6）PBS洗2-3次各5分鐘 （7）滴加正常山羊血清封閉液，室溫20分鐘，甩去多余液體（8）滴加Ι抗50μl,室溫靜置1小時或4℃過夜或37℃1小時（9）4℃過夜后需在37℃復溫45分鐘（10）PBS洗3次每次2分鐘
（11）滴加Ⅱ抗45-50μl,室溫靜置或37℃1小時（12）Ⅱ抗中可加入0.05%的
tween-20.（13）PBS洗3次各5分鐘（14）DAB顯色5-10分鐘，在顯微鏡下掌握染色程度 （15）PBS或自來水沖洗10分鐘 （16）蘇木精復染2分鐘，鹽酸酒精分化（17）自來水沖洗10-15分鐘 （18）脫水、透明、封片、鏡檢。
SABC法
（1） 脫蠟、水化 （2）PBS洗2次各5分鐘 （3）用蒸餾水或PBS配制新鮮的3%H2O2，室溫封閉5-10分鐘，用蒸餾水洗3次（4）抗原修復
（5） PBS洗5分鐘 （6）滴加正常山羊血清封閉液，室溫20分鐘，甩去多余液體（7）滴加Ι抗50μl,室溫靜置1小時或4℃過夜或37℃1小時
（8）PBS洗3次各2分鐘（9）滴加*化Ⅱ抗，20℃-37℃ 20分鐘
（10）PBS洗3次各2分鐘 （11）滴加試劑SABC 20℃-30℃ 20分鐘
（12） PBS洗4次各5分鐘 （13） DAB顯色：試劑盒或自配顯色劑顯色
（14）脫水、透明、封片、鏡檢。

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