HPA002532
抗C10orf2抗体 ウサギ宿主抗体
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
別名:
抗PEO1抗体, 抗T7 gp4-様タンパク質( ミトコンドリア内ヌクレオシド局在)抗体, 抗T7-様ミトコンドリアDNA ヘリカーゼ抗体, 抗Twinkleタンパク質、ミトコンドリア前駆体抗体, 抗進行性外眼筋麻痺 1タンパク質抗体
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About This Item
UNSPSCコード:
12352203
Human Protein Atlas Number:
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由来生物
rabbit
品質水準
結合体
unconjugated
抗体製品の状態
affinity isolated antibody
抗体製品タイプ
primary antibodies
クローン
polyclonal
製品種目
Prestige Antibodies® Powered by Atlas Antibodies
形状
buffered aqueous glycerol solution
化学種の反応性
human
テクニック
immunohistochemistry: 1:200- 1:500
免疫原配列
SKAPEFEDSEEVRRIWNRAIPLWELPDQEEVQLADTMFGLTKVTDDTLKRFSVRYLRPARSLVFPWFSPGGSGLRGLKLLEAKCQGDGVSYEETTIPRPSAYHNLFGLPLISRRDAEVVLTSRELDSLALNQSTG
輸送温度
wet ice
保管温度
−20°C
ターゲットの翻訳後修飾
unmodified
遺伝子情報
human ... C10orf2(56652)
免疫原
twinkle mtDNA helicase recombinant protein epitope signature tag (PrEST)
アプリケーション
Prestige抗体®は、Human Proteome Resource(HPR)プロジェクト(www.proteinatlas.org)によって開発・実証されたAtlas抗体です。抗体はすべて、数百の正常組織・疾病組織に対する免疫組織染色試験を行っています。これらの染色画像はHuman Protein Atlas(HPA)サイトで[Image Gallery]リンクをクリックするとご覧いただけます。さらに、ほとんどのPrestige抗体はプロテインアレイおよびウェスタンブロッティングの試験を行っています。試験のプロトコールおよびPrestige抗体、HPAに関する情報はsigma.com/prestigeをご覧ください。
生物化学的／生理学的作用
C10orf2, a twinkle protein, encodes a mitochondrial primase-helicase essential for the mitochondrial DNA replication. It has been reported that it can inefficiently unwinds intermolecular and intramolecular G-quadruplex DNA structure. Recessive C10orf2 mutation causes infantile-onset spinocerebellar ataxia (IOSCA) with sensorimotor polyneuropathy and myopathy. It is also associated with the hepatocerebral syndrome, and autosomal dominant PEO (adPEO).
特徴および利点
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
関連事項
Corresponding Antigen APREST86625
物理的形状
PBS溶液 (pH 7.2, 40%グリセロ-ル, 0.02%アジ化ナトリウム含有)
法的情報
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
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保管分類コード
10 - Combustible liquids
WGK
WGK 1
引火点(°F)
Not applicable
引火点(℃)
Not applicable
個人用保護具 (PPE)
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
試験成績書（COA）
製品のロット番号・バッチ番号を入力して、試験成績書（COA） を検索できます。ロット番号・バッチ番号は、製品ラベルに「Lot」または「Batch」に続いて記載されています。
Sanjay Kumar Bharti et al.
The Journal of biological chemistry, 289(43), 29975-29993 (2014-09-07)
Mitochondrial DNA deletions are prominent in human genetic disorders, cancer, and aging. It is thought that stalling of the mitochondrial replication machinery during DNA synthesis is a prominent source of mitochondrial genome instability; however, the precise molecular determinants of defective
Mi-Hyun Park et al.
Neurogenetics, 15(3), 171-182 (2014-05-13)
Recessive mutations in chromosome 10 open reading frame 2 (C10orf2) are relevant in infantile-onset spinocerebellar ataxia (IOSCA). In this study, we investigated the causative mutation in a Korean family with combined phenotypes of IOSCA, sensorimotor polyneuropathy, and myopathy. We investigated
Hiroyuki Morino et al.
Neurology, 83(22), 2054-2061 (2014-10-31)
To identify the genetic cause in 2 families of progressive ataxia, axonal neuropathy, hyporeflexia, and abnormal eye movements, accompanied by progressive hearing loss and ovarian dysgenesis, with a clinical diagnosis of Perrault syndrome. Whole-exome sequencing was performed to identify causative
Viktoriya Peeva et al.
Nature communications, 9(1), 1727-1727 (2018-05-02)
Emerging gene therapy approaches that aim to eliminate pathogenic mutations of mitochondrial DNA (mtDNA) rely on efficient degradation of linearized mtDNA, but the enzymatic machinery performing this task is presently unknown. Here, we show that, in cellular models of restriction
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