概述

  • 产品名称Anti-ATP6V0A2抗体
    参阅全部 ATP6V0A2 一抗
  • 描述
    兔多克隆抗体to ATP6V0A2
  • 经测试应用适用于: IHC-P, ICC/IF, WBmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat, Horse, Cow, Cat, Dog, Pig
  • 免疫原

    Synthetic peptide corresponding to a region within N terminal amino acids 71-120 (INRADIPLPE GEASPPAPPL KQVLEMQEQL QKLEVELREV TKNKEKLRKN) of Human ATP6V0A2 (NP_036595)

  • 阳性对照
    • HeLa cell lysate

性能

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应用

Our Abpromise guarantee covers the use of ab82638 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
IHC-P Use a concentration of 5 µg/ml.
ICC/IF 1/250.
WB Use a concentration of 1 µg/ml. Predicted molecular weight: 98 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.

靶标

  • 功能Part of the proton channel of V-ATPases. Essential component of the endosomal pH-sensing machinery. May play a role in maintaining the Golgi functions, such as glycosylation maturation, by controlling the Golgi pH.
  • 疾病相关Defects in ATP6V0A2 are the cause of cutis laxa autosomal recessive type 2A (ARCL2A) [MIM:219200]. An autosomal recessive disorder characterized by an excessive congenital skin wrinkling, a large fontanelle with delayed closure, a typical facial appearance with downslanting palpebral fissures, a general connective tissue weakness, and varying degrees of growth and developmental delay and neurological abnormalities. Some affected individuals develop seizures and mental deterioration later in life, whereas the skin phenotype tends to become milder with age. At the molecular level, an abnormal glycosylation of serum proteins is observed in many cases.
    Defects in ATP6V0A2 are a cause of wrinkly skin syndrome (WSS) [MIM:278250]. WSS is rare autosomal recessive disorder characterized by wrinkling of the skin of the dorsum of the hands and feet, an increased number of palmar and plantar creases, wrinkled abdominal skin, multiple musculoskeletal abnormalities, microcephaly, growth failure and developmental delay.
  • 序列相似性Belongs to the V-ATPase 116 kDa subunit family.
  • 翻译后修饰Phosphorylated upon DNA damage, probably by ATM or ATR.
  • 细胞定位Cell membrane. Endosome membrane. In kidney proximal tubules, also detected in subapical vesicles.
  • Information by UniProt
  • 数据库链接
  • 别名
    • a2 antibody
    • A2V ATPase antibody
    • ARCL antibody
    • ATP6a2 antibody
    • ATP6N1D antibody
    • ATP6V0A2 antibody
    • ATPase, H+ transporting, lysosomal V0 subunit a isoform 2 antibody
    • ATPase, H+ transporting, lysosomal V0 subunit a2 antibody
    • Infantile malignant osteopetrosis antibody
    • J6B7 antibody
    • Lysosomal H(+) transporting ATPase V0 subunit a2 antibody
    • Lysosomal H(+)-transporting ATPase V0 subunit a2 antibody
    • regeneration and tolerance factor antibody
    • Stv1 antibody
    • TJ6 antibody
    • TJ6M antibody
    • TJ6s antibody
    • V ATPase 116 kDa isoform a2 antibody
    • V type proton ATPase 116 kDa subunit a antibody
    • V type proton ATPase 116 kDa subunit a isoform 2 antibody
    • V-ATPase 116 kDa isoform a2 antibody
    • V-type proton ATPase 116 kDa subunit a isoform 2 antibody
    • Vacuolar proton translocating ATPase 116 kDa subunit a antibody
    • Vacuolar proton translocating ATPase 116 kDa subunit a isoform 2 antibody
    • Vph1 antibody
    • VPP2_HUMAN antibody
    • WSS antibody
    see all

Anti-ATP6V0A2 antibody 图像

  • Immunohistochemistry with Human kidney tissue at an antibody concentration of 5.0ug/ml using anti-ATP6V0A2 antibody (ab82638)

  • Application: IHC/Immunofluorescence. Species+tissue/cell type:A: untransfected HeLa cells. B:mATP6V0A2-FLAG transfected HeLa cells. C: mATP6V0A2 (partial) transfected HeLa cells. Primary antibody dilution: 1:250. Secondary antibody: Anti-rabbit AlexaFluor 488. Secondary antibody dilution: 1:1000
  • Anti-ATP6V0A2 antibody (ab82638) at 1 µg/ml + HeLa cell lysate at 10 µg

    Secondary
    HRP-conjugated anti-Rabbit IgG at 1/50000 dilution

    Predicted band size : 98 kDa
    Observed band size : 88 kDa (why is the actual band size different from the predicted?)
    Additional bands at : 45 kDa,50 kDa. We are unsure as to the identity of these extra bands.
    Gel concentration 12%
  • ab82638 at 5 µg/ml staining ATP6V0A2 in Human small intestine tissue by Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections).
  • ab82638 at 5 µg/ml staining ATP6V0A2 in Human small intestine tissue by Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections).

Anti-ATP6V0A2 antibody (ab82638)参考文献

ab82638 has not yet been referenced specifically in any publications.

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