Signal Transduction Antibodies 3

Anti-ATP6V1B1 Antibody (CAB6876)

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SKU:
CAB6876
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-ATP6V1B1 Antibody
Antibody SKU:CAB6876
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 ATP6V1B1 (NP_001683.2).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse
Positive Samples:BT-474, MCF7, HepG2, Mouse testis, Mouse kidney
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human ATP6V1B1 (NP_001683.2).
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:MAME IDSR PGGL PGSS CNLG AARE HMQA VTRN YITH PRVT YRTV CSVN GPLV VLDR VKFA QYAE IVHF TLPD GTQR SGQV LEVA GTKA IVQV FEGT SGID ARKT TCEF TGDI LRTP VSED MLGR VFNG SGKP IDKG PVVM AEDF LDIN GQPI NPHS RIYP EEMI QTGI SPID VMNS IARG QKIP IFSA AGLP HNEI AAQI CRQA GLVK KSKA VLDY HDDN FAIV FAAM GVNM ETAR FFKS DFEQ NGTM GNVC LFLN LAND PTIE RIIT PRLA LTTA EFLA
Gene ID:525
Uniprot:P15313
Cellular Location:Endomembrane system, Peripheral membrane protein
Calculated MW:56kDa
Observed MW:57kDa
Synonyms:ATP6V1B1, ATP6B1, RTA1B, VATB, VMA2, VPP3
Background:This gene encodes a component of vacuolar ATPase (V-ATPase), a multisubunit enzyme that mediates acidification of eukaryotic intracellular organelles. V-ATPase dependent organelle 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. Additional isoforms of many of the V1 and V0 subunit proteins are encoded by multiple genes or alternatively spliced transcript variants. This encoded protein is one of two V1 domain B subunit isoforms and is found in the kidney. Mutations in this gene cause distal renal tubular acidosis associated with sensorineural deafness.
UniProt Protein Function:ATP6V1B1: Non-catalytic subunit of the peripheral V1 complex of vacuolar ATPase. V-ATPase is responsible for acidifying a variety of intracellular compartments in eukaryotic cells. Defects in ATP6V1B1 are the cause of distal renal tubular acidosis with deafness (dRTA-D). Inheritance is autosomal recessive. Patients with recessive dRTA are severely affected, presenting with either acute illness or growth failure at a young age, and bilateral sensorineural deafness. Other features include low serum K(+) due to renal potassium wasting, and elevated urinary calcium. If untreated, this acidosis may result in dissolution of bone, leading to osteomalacia and rickets. Renal deposition of calcium salts (nephrocalcinosis) and renal stone formation commonly occur. Belongs to the ATPase alpha/beta chains family.
UniProt Protein Details:

Protein type:Energy Metabolism - oxidative phosphorylation; EC 3.6.3.14; Hydrolase

Chromosomal Location of Human Ortholog: 2p13.1

Cellular Component: apical plasma membrane; basolateral plasma membrane; cytoplasm; cytosol; lateral plasma membrane; microvillus; vacuolar proton-transporting V-type ATPase complex

Molecular Function:hydrogen ion transmembrane transporter activity

Biological Process: calcium ion homeostasis; excretion; inner ear morphogenesis; insulin receptor signaling pathway; ossification; pH reduction; proton transport; regulation of pH; sensory perception of sound; transferrin transport

Disease: Renal Tubular Acidosis, Distal, With Progressive Nerve Deafness

NCBI Summary:This gene encodes a component of vacuolar ATPase (V-ATPase), a multisubunit enzyme that mediates acidification of eukaryotic intracellular organelles. V-ATPase dependent organelle 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. Additional isoforms of many of the V1 and V0 subunit proteins are encoded by multiple genes or alternatively spliced transcript variants. This encoded protein is one of two V1 domain B subunit isoforms and is found in the kidney. Mutations in this gene cause distal renal tubular acidosis associated with sensorineural deafness. [provided by RefSeq, Jul 2008]
UniProt Code:P15313
NCBI GenInfo Identifier:215274116
NCBI Gene ID:525
NCBI Accession:P15313.3
UniProt Secondary Accession:P15313,Q53FY0, Q6P4H6,
UniProt Related Accession:P15313
Molecular Weight:56,833 Da
NCBI Full Name:V-type proton ATPase subunit B, kidney isoform
NCBI Synonym Full Names:ATPase H+ transporting V1 subunit B1
NCBI Official Symbol:ATP6V1B1  
NCBI Official Synonym Symbols:VATB; VMA2; VPP3; RTA1B; ATP6B1  
NCBI Protein Information:V-type proton ATPase subunit B, kidney isoform
UniProt Protein Name:V-type proton ATPase subunit B, kidney isoform
UniProt Synonym Protein Names:Endomembrane proton pump 58 kDa subunit; Vacuolar proton pump subunit B 1
Protein Family:V-type proton ATPase
UniProt Gene Name:ATP6V1B1  
UniProt Entry Name:VATB1_HUMAN
Anti-ATP6V1B1 Antibody (CAB6876)Western blot analysis of extracts of various cell lines, using ATP6V1B1 antibody (CAB6876) 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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