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檢測Anti-Twinkle/ATXN8抗體,共濟失調蛋白8抗體簡單介紹：

產品別名：Ataxin 8; Ataxin8; ATXN 8; ATXN8; C10 orf2; C10orf 2; C10orf2; Chromosome 10 open reading frame 2; IOSCA; PEO 1; PEO; PEO1; PEOA3; Progressive external ophthalmoplegia 1 protein; SANDO; SCA 8; SCA8; T7 gp4 like protein with intramitochondrial nucleoid localization; T7 like mitochondrial DNA helicase; Twinkle protein, mitochondrial; TWINL.

產品用途：科研實驗

產品編號：BYK-11775R

性 狀：Lyophilized or Liquid

濃 度：1mg/1ml

亞 型：IgG

貯 存：貯存于-20℃.

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）

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

檢測Anti-Twinkle/ATXN8抗體,共濟失調蛋白8抗體產品說明：Twinkle, also known as PEO1 (Progressive external ophthalmoplegia 1 protein), PEOA3, SANDO or TWINL, is a mitochondrial protein that functions as a 5’-3’ nucleotide-dependent DNA helicase. Colocalized with mtDNA (mitochondrial DNA) in mitochondrial nucleoids, Twinkle is important in the metabolism and maintenance of mtDNA, playing a crucial role in the regulation of mtDNA copy numbers. Twinkle is expressed at high levels in testis, pancreas and skeletal muscle and exists as three isoforms due to alternative splicing events. Defects in the gene encoding Twinkle are the cause of two conditions: progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal dominant 3 (PEOA3) and sensory ataxic neuropathy dysarthria and ophthalmoparesis (SANDO). PEOA3 is characterized by ptosis and weak muscles, while SANDO is characterized by ophthalmoparesis, dysarthria and sensory ataxic neuropathies.

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.
連續使用時4°C存儲，保質期六個月補體C1qα鏈多肽抗體*存儲時建議分裝為10ul以上小包裝-20°C存儲，并避免反復凍融，保質期一年。

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