ELISA/assay

Human V-type proton ATPase 116 kDa subunit a isoform 2, ATP6V0A2 ELISA Kit

MBS911862

48-Strip-Wells

510 USD

ELISA Kit

Human V-type proton ATPase 116 kDa subunit a isoform 2, ATP6V0A2 ELISA Kit

ATPase, H+ transporting, lysosomal V0 subunit a2

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

V-type proton ATPase 116 kDa subunit a isoform 2; V-type proton ATPase 116 kDa subunit a isoform 2; 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; ATPase, H+ transporting, lysosomal V0 subunit a2; Lysosomal H(+)-transporting ATPase V0 subunit a2; TJ6; Vacuolar proton translocating ATPase 116 kDa subunit a isoform 2

ATP6V0A2

ATP6V0A2; ATP6V0A2; A2; RTF; TJ6; WSS; ARCL; J6B7; STV1; TJ6M; TJ6S; VPH1; ARCL2A; ATP6A2; ATP6N1D; V-ATPase 116 kDa isoform a2

VPP2_HUMAN

Human

856

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.

Samples: Serum, plasma, tissue homogenates. Assay Type: Sandwich. Detection Range: 31.25 pg/ml -2000 pg/ml. Sensitivity: 7.81 pg/ml.

Intra-assay Precision: Intra-assay Precision (Precision within an assay): CV%<8%. Three samples of known concentration were tested twenty times on one plate to assess. Inter-assay Precision: Inter-assay Precision (Precision between assays): CV%<10%. Three samples of known concentration were tested in twenty assays to assess.

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.

42741679

NP_036595.2

NM_012463.3

Q9Y487

98,082 Da

Collecting Duct Acid Secretion Pathway (147586); Collecting Duct Acid Secretion Pathway (147560); Epithelial Cell Signaling In Helicobacter Pylori Infection Pathway (83102); Epithelial Cell Signaling In Helicobacter Pylori Infection Pathway (515); Insulin Receptor Recycling Pathway (106438); Iron Uptake And Transport Pathway (187191); Lysosome Pathway (99052); Lysosome Pathway (96865); Oxidative Phosphorylation Pathway (82942); Oxidative Phosphorylation Pathway (303)

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

48-Strip-Wells

510 USD

96-Strip-Wells

725

5x96-Strip-Wells

2565

10x96-Strip-Wells

4800