Rabbit anti-Human ATP6V0A2 Polyclonal Antibody | anti-ATP6V0A2 antibody

ATP6V0A2 antibody

Cat. Num. AAA9404043

• Gene Names: ATP6V0A2; A2; RTF; TJ6; WSS; ARCL; J6B7; STV1; TJ6M; TJ6S; VPH1; ARCL2A; ATP6A2; ATP6N1D
• Reactivity: Human
• Applications: Western Blot, Immunohistochemistry, Immunofluorescence
• Purity: Purified by antigen-affinity chromatography.
• Synonyms: ATP6V0A2; Polyclonal Antibody; ATP6V0A2 antibody; anti-ATP6V0A2 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Purity/Purification: Purified by antigen-affinity chromatography.
• Form/Format: Supplied in 0.1M Tris-buffered saline with 10% Glycerol (pH7.0). 0.01% Thimerosal was added as a preservative.
• Sequence Length: 856

• Applicable Applications for anti-ATP6V0A2 antibody: Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)
• Application Notes: Western blotting: 1:500-1:3000; Immunohistochemistry: 1:100-1:500; Immunofluorescence: 1:100-1:200
• Immunogen Type: Recombinant protein
• Immunogen Description: Recombinant protein fragment contain a sequence corresponding to a region within amino acids 156 and 404 of ATP6V0A2
• Target Name: ATP6V0A2
• Preparation and Storage: Store at -20 degree C for long term preservation (recommended). Store at 4 degree C for short term use.

Testing Data

(Sample (30 ug of whole cell lysate) A: 293T B: A431 C: H1299 7.5% SDS PAGE Primary antibody diluted at 1: 10000)

Immunohistochemistry (IHC)

(Immunohistochemical analysis of paraffin-embedded DLD1 xenograft, using ATP6V0A2 antibody at 1: 500 dilution.)

Immunofluorescence (IF)

(Confocal immunofluorescence analysis (Olympus FV10i) of methanol-fixed A431, using ATP6V0A2 antibody (Green) at 1: 500 dilution and alpha-tubulin antibody (Red) at 1: 500.)

Related Product Information for anti-ATP6V0A2 antibody

The multisubunit vacuolar-type proton pump (H(+)-ATPase or V-ATPase) is essential for acidification of diverse cellular components, including endosomes, lysosomes, clathrin-coated vesicles, secretory vesicles, and chromaffin granules, and it is found at high density in the plasma membrane of certain specialized cells. H(+)-ATPases are comprised of a peripheral V(1) domain and an integral membrane V(0) domain; ATP6V0A2 is a component of the V(0) domain (Smith et al., 2003 [PubMed 14580332]).[supplied by OMIM]

Product Categories/Family for anti-ATP6V0A2 antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 42741679
• NCBI GeneID: 23545
• NCBI Accession #: NP_036595
• NCBI GenBank Nucleotide #: NM_012463.3
• Molecular Weight: 98,082 Da
• NCBI Official Full Name: V-type proton ATPase 116 kDa subunit a isoform 2
• NCBI Official Synonym Full Names: ATPase, H+ transporting, lysosomal V0 subunit a2
• NCBI Official Symbol: ATP6V0A2
• NCBI Official Synonym Symbols: A2; RTF; TJ6; WSS; ARCL; J6B7; STV1; TJ6M; TJ6S; VPH1; ARCL2A; ATP6A2; ATP6N1D
• NCBI Protein Information: V-type proton ATPase 116 kDa subunit a isoform 2
• UniProt Protein Name: V-type proton ATPase 116 kDa subunit a isoform 2
• Protein Family: V-type proton ATPase
• UniProt Gene Name: ATP6V0A2
• UniProt Synonym Gene Names: V-ATPase 116 kDa isoform a2
• UniProt Entry Name: VPP2_HUMAN

NCBI Description

The protein encoded by this gene is a subunit of the vacuolar ATPase (v-ATPase), an heteromultimeric enzyme that is present in intracellular vesicles and in the plasma membrane of specialized cells, and which is essential for the acidification of diverse cellular components. V-ATPase is comprised of a membrane peripheral V(1) domain for ATP hydrolysis, and an integral membrane V(0) domain for proton translocation. The subunit encoded by this gene is a component of the V(0) domain. Mutations in this gene are a cause of both cutis laxa type II and wrinkly skin syndrome. [provided by RefSeq, Jul 2009]

Uniprot Description

ATP6V0A2: 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. Defects in ATP6V0A2 are the cause of cutis laxa autosomal recessive type 2A (ARCL2A). 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). 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. Belongs to the V-ATPase 116 kDa subunit family.

Protein type: Membrane protein, integral; Transporter, ion channel; Membrane protein, multi-pass; Transporter, iron; Transporter; Energy Metabolism - oxidative phosphorylation

Chromosomal Location of Human Ortholog: 12q24.31

Cellular Component: phagocytic vesicle membrane; focal adhesion; lysosomal membrane; cytoplasm; acrosome; plasma membrane; integral to membrane; endosome membrane; vacuolar proton-transporting V-type ATPase complex

Molecular Function: protein binding; hydrogen ion transporting ATPase activity, rotational mechanism; ATPase binding

Biological Process: vacuolar acidification; interaction with host; ATP synthesis coupled proton transport; cellular iron ion homeostasis; ATP hydrolysis coupled proton transport; insulin receptor signaling pathway; transferrin transport; immune response; transmembrane transport

Disease: Cutis Laxa, Autosomal Recessive, Type Iia; Wrinkly Skin Syndrome