V-type proton ATPase 116 kDa subunit a isoform 2; V-ATPase 116 kDa isoform a2; Lysosomal H(+)-transporting ATPase V0 subunit a2; TJ6; Vacuolar proton translocating ATPase 116 kDa subunit a isoform 2; ATP6V0A2

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

Polyclonal

IgG

Rabbit

>95%,Protein G purified

Liquid

Shipped at 4 degree C. Upon delivery, aliquot and store at -20 degree C or -80 degree C.

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

Small volumes of anti-ATP6V0A2 antibody 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.

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.

ELISA (EIA)

98,082 Da

ATPase H+ transporting V0 subunit a2

V-type proton ATPase 116 kDa subunit a isoform 2

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

Chromosomal Location of Human Ortholog: 12q24.31

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

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

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

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

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