Signal Transduction Antibodies 3

Anti-ATP6V0A4 Antibody (CAB7591)

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SKU:
CAB7591
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Signal Transduction

Description

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Antibody Name:Anti-ATP6V0A4 Antibody
Antibody SKU:CAB7591
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human ATP6V0A4 (NP_065683.2).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse
Positive Samples:A375, Mouse kidney
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human ATP6V0A4 (NP_065683.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:MVSV FRSE EMCL SQLF LQVE AAYC CVAE LGEL GLVQ FKDL NMNV NSFQ RKFV NEVR RCES LERI LRFL EDEM QNEI VVQL LEKS PLTP LPRE MITL ETVL EKLE GELQ EANQ NQQA LKQS FLEL TELK YLLK KTQD FFET ETNL ADDF FTED TSGL LELK AVPA YMTG KLGF IAGV INRE RMAS FERL LWRI CRGN VYLK FSEM DAPL EDPV TKEE IQKN IFII FYQG EQLR QKIK KICD GFRA TVYP CPEP AVER REML ESVN VRLE DLIT VITQ TESH
Gene ID:50617
Uniprot:Q9HBG4
Cellular Location:Apical cell membrane, Multi-pass membrane protein
Calculated MW:96kDa
Observed MW:70-90kDa
Synonyms:ATP6V0A4, A4, ATP6N1B, ATP6N2, RDRTA2, RTA1C, RTADR, STV1, VPH1, VPP2
Background:This gene encodes a component of vacuolar ATPase (V-ATPase), a multisubunit enzyme that mediates acidification of intracellular compartments of eukaryotic cells. V-ATPase dependent acidification is necessary for such intracellular processes as protein sorting, zymogen activation, receptor-mediated endocytosis, and synaptic vesicle proton gradient generation. V-ATPase is composed of a cytosolic V1 domain and a transmembrane V0 domain. The V1 domain consists of three A and three B subunits, two G subunits plus the C, D, E, F, and H subunits. The V1 domain contains the ATP catalytic site. The V0 domain consists of five different subunits: a, c, c', c'', and d. This gene is one of four genes in man and mouse that encode different isoforms of the a subunit. Alternatively spliced transcript variants encoding the same protein have been described. Mutations in this gene are associated with renal tubular acidosis associated with preserved hearing.
UniProt Protein Function:ATP6V0A4: Part of the proton channel of the V-ATPase that is involved in normal vectorial acid transport into the urine by the kidney. Defects in ATP6V0A4 are the cause of distal renal tubular acidosis with preserved hearing (RTADR). RTADR is an autosomal recessive form of distal renal tubular acidosis (dRTA), a group of disorders characterized by functional failure of alpha- intercalated cells of the cortical collecting duct of the distal nephron, where vectorial proton transport is required for urinary acidification. Functional failure of alpha-intercalated cells results in metabolic acidosis accompanied by disturbances of potassium balance, urinary calcium solubility, bone physiology and growth. Belongs to the V-ATPase 116 kDa subunit family.
UniProt Protein Details:

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

Chromosomal Location of Human Ortholog: 7q34

Cellular Component: phagocytic vesicle membrane; brush border membrane; apical part of cell; lysosomal membrane; apical plasma membrane; integral to membrane; plasma membrane; endosome membrane; vacuolar proton-transporting V-type ATPase complex; endosome

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; ossification; proton transport; sensory perception of sound; cellular iron ion homeostasis; ATP hydrolysis coupled proton transport; insulin receptor signaling pathway; transferrin transport; regulation of pH; excretion; transmembrane transport

Disease: Renal Tubular Acidosis, Distal, Autosomal Recessive

NCBI Summary:This gene encodes a component of vacuolar ATPase (V-ATPase), a multisubunit enzyme that mediates acidification of intracellular compartments of eukaryotic cells. V-ATPase dependent acidification is necessary for such intracellular processes as protein sorting, zymogen activation, receptor-mediated endocytosis, and synaptic vesicle proton gradient generation. V-ATPase is composed of a cytosolic V1 domain and a transmembrane V0 domain. The V1 domain consists of three A and three B subunits, two G subunits plus the C, D, E, F, and H subunits. The V1 domain contains the ATP catalytic site. The V0 domain consists of five different subunits: a, c, c', c'', and d. This gene is one of four genes in man and mouse that encode different isoforms of the a subunit. Alternatively spliced transcript variants encoding the same protein have been described. Mutations in this gene are associated with renal tubular acidosis associated with preserved hearing. [provided by RefSeq, Jul 2008]
UniProt Code:Q9HBG4
NCBI GenInfo Identifier:85386053
NCBI Gene ID:50617
NCBI Accession:NP_065683.2
UniProt Secondary Accession:Q9HBG4,Q32M47, A4D1R4, A8KA80,
UniProt Related Accession:Q9HBG4
Molecular Weight:96,386 Da
NCBI Full Name:V-type proton ATPase 116 kDa subunit a isoform 4
NCBI Synonym Full Names:ATPase, H+ transporting, lysosomal V0 subunit a4
NCBI Official Symbol:ATP6V0A4  
NCBI Official Synonym Symbols:A4; STV1; VPH1; VPP2; RTA1C; RTADR; ATP6N2; RDRTA2; ATP6N1B  
NCBI Protein Information:V-type proton ATPase 116 kDa subunit a isoform 4; V-ATPase 116 kDa; vacuolar proton pump, subunit 2; vacuolar proton pump 116 kDa accessory subunit; vacuolar proton translocating ATPase 116 kDa subunit a kidney isoform; H(+)-transporting two-sector ATPase, noncatalytic accessory protein 1B; ATPase, H+ transporting, lysosomal (vacuolar proton pump) non-catalytic accessory protein 1B; ATPase, H+ transporting, lysosomal (vacuolar proton pump) non-catalytic accessory protein 2 (38kD)
UniProt Protein Name:V-type proton ATPase 116 kDa subunit a isoform 4
UniProt Synonym Protein Names:Vacuolar proton translocating ATPase 116 kDa subunit a isoform 4; Vacuolar proton translocating ATPase 116 kDa subunit a kidney isoform
Protein Family:V-type proton ATPase
UniProt Gene Name:ATP6V0A4  
UniProt Entry Name:VPP4_HUMAN
Anti-ATP6V0A4 Antibody (CAB7591)Western blot analysis of extracts of various cell lines, using ATP6V0A4 antibody (CAB7591) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.
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