ATPase, H+ transporting, lysosomal V0 subunit a2, ELISA Kit

Human V-type proton ATPase 116 kDa subunit a isoform 2 (ATP6V0A2) ELISA kit; A2; ARCL; ATP6A2; ATP6N1D; J6B7; RTF; STV1; TJ6; TJ6M; TJ6S; VPH1; WSS; A2V-ATPase; ATPase; H+ transporting; lysosomal V0 subunit A2; regeneration and tolerance factor; v-ATPase 116 kDa; v-type proton A; ATPase; H+ transporting; lysosomal V0 subunit a2

For Research Use Only. Not for use in diagnostic procedures.

This assay has high sensitivity and excellent specificity for detection of human ATP6V0A2. No significant cross-reactivity or interference between human ATP6V0A2 and analogues was observed.

Unopened test kits should be stored at 2 to 8 degree C upon receipt. Please refer to pdf manual for further storage instructions.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.

Small volumes of ATP6V0A2 elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS911862 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the ATPase, H+ transporting, lysosomal V0 subunit a2 (ATP6V0A2) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing ATP6V0A2. The ELISA analytical biochemical technique of the MBS911862 kit is based on ATP6V0A2 antibody-ATP6V0A2 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect ATP6V0A2 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, ATP6V0A2. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Principle of the Assay: This assay employs the quantitative sandwich enzyme immunoassay technique. Antibody specific for ATP6V0A2 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any ATP6V0A2 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for ATP6V0A2 is added to the wells. After washing, avidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of ATP6V0A2 bound in the initial step. The color development is stopped and the intensity of the color is measured.

98,082 Da

ATPase, H+ transporting, lysosomal V0 subunit a2

V-type proton ATPase 116 kDa subunit a isoform 2; A2V-ATPase; v-ATPase 116 kDa; regeneration and tolerance factor; v-type proton ATPase 116 kDa subunit a; lysosomal H(+)-transporting ATPase V0 subunit a2; vacuolar proton translocating ATPase 116 kDa subunit a

V-type proton ATPase 116 kDa subunit a isoform 2

V-ATPase 116 kDa isoform a2  [Similar Products]

VPP2_HUMAN

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]

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

Chromosomal Location of Human Ortholog: 12q24.31

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

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

Biological Process: interaction with host; vacuolar acidification; 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

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