Anti-ATP6V0A4 antibody (AA40262)

$187.00


Alternative names V-type proton ATPase 116 kDa subunit a isoform 4, V-ATPase 116 kDa isoform a4, Vacuolar proton translocating ATPase 116 kDa subunit a isoform 4, Vacuolar proton translocating ATPase 116 kDa subunit a kidney isoform, ATP6V0A4, ATP6N1B, ATP6N2, VPP4_HUMAN, Q9HBG4, 50617
Source 840
Species Reactivity Human
Applications IHC, WB
Host Rabbit
Class Polyclonal
Conjugated Unconjugated


Description

Product name Anti-ATP6V0A4 antibody (AA40262)
Catalog number AA40262
Brand family Ango
Product type Primary Antibody
Antibody Alternative Names V-type proton ATPase 116 kDa subunit a isoform 4 antibody, V-ATPase 116 kDa isoform a4 antibody, Vacuolar proton translocating ATPase 116 kDa subunit a isoform 4 antibody, Vacuolar proton translocating ATPase 116 kDa subunit a kidney isoform antibody, ATP6V0A4 antibody, ATP6N1B antibody, ATP6N2 antibody, VPP4_HUMAN antibody


Biological Information

Concentration 1 mg/ml
Isotype IgG
Purification method Purified by affinity chromatography


Target

Target Name ATP6V0A4
SwissProt ID Link Q9HBG4
Function Part of the proton channel of the V-ATPase that is involved in normal vectorial acid transport into the urine by the kidney.
Subcellular Location Apical cell membrane; Multi-pass membrane protein. Present at high density almost exclusively on the apical surface of alpha-intercalated cells in the cortical collecting ducts of the distal nephron.
Involvement in Disease Renal tubular acidosis, distal, autosomal recessive: An autosomal recessive disease characterized by reduced ability to acidify urine, variable hyperchloremic hypokalemic metabolic acidosis, nephrocalcinosis, and nephrolithiasis. It is due to functional failure of alpha-intercalated cells of the cortical collecting duct of the distal nephron, where vectorial proton transport is required for urinary acidification. The disease is caused by mutations affecting the gene represented in this entry.
Tissue Specificity Expressed in adult and fetal kidney. Found in the inner ear.
Subunit Structure The V-ATPase is a heteromultimeric enzyme composed of at least thirteen different subunits. It has a membrane peripheral V1 sector for ATP hydrolysis and an integral V0 for proton translocation. The V1 sector comprises subunits A-H, whereas V0 includes subunits a, d, c, c', and c''.
Sequence Similarities Belongs to the V-ATPase 116 kDa subunit family.


Poduct Presentation

Form Liquid
Preservative 0.02% (w/v) sodium azide
Storage buffer PBS with 50% glycerol, pH 7.3


Storage and Shipping Information

Storage Store at -20 C. Avoid multiple freeze/thaw cycles.