Product nameAnti-ATP6V0A2 antibody
See all ATP6V0A2 primary antibodies
DescriptionRabbit polyclonal to ATP6V0A2
Tested applicationsSuitable for: WB, IHC-Pmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat, Cow
Recombinant fragment corresponding to a region within amino acids 156-404 of Human ATP6V0A2 (NP_036595).
- 293T, A431 and H1299 whole cell lysates; DLD1 xenograft; HeLa, HepG2, MOLT4 and Raji cell lysates
Storage instructionsShipped at 4°C. Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
Storage bufferpH: 7.00
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 1.21% Tris, 0.75% Glycine, 20% Glycerol
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab96803 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/500 - 1/3000. Predicted molecular weight: 98 kDa.|
|IHC-P||1/100 - 1/500.|
FunctionPart 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.
Involvement in diseaseDefects 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.
Sequence similaritiesBelongs to the V-ATPase 116 kDa subunit family.
modificationsPhosphorylated upon DNA damage, probably by ATM or ATR.
Cellular localizationCell membrane. Endosome membrane. In kidney proximal tubules, also detected in subapical vesicles.
- Information by UniProt
- a2 antibody
- A2V ATPase antibody
- ARCL antibody
All lanes : Anti-ATP6V0A2 antibody (ab96803) at 1/10000 dilution
Lane 1 : 293T whole cell lysate
Lane 2 : A431 whole cell lysate
Lane 3 : H1299 whole cell lysate
Lysates/proteins at 30 µg per lane.
Predicted band size: 98 kDa
7.5% SDS PAGE
ab96803, at 1/500 dilution, staining ATP6V0A2 in paraffin-embedded DLD1 xenograft by Immunohistochemistry.
This product has been referenced in:
- Kissing S et al. Disruption of the vacuolar-type H+-ATPase complex in liver causes MTORC1-independent accumulation of autophagic vacuoles and lysosomes. Autophagy 13:670-685 (2017). Read more (PubMed: 28129027) »
- Tripathi M et al. Hyperhomocysteinemia causes ER stress and impaired autophagy that is reversed by Vitamin B supplementation. Cell Death Dis 7:e2513 (2016). Read more (PubMed: 27929536) »