Anti-ATP6V1B1 Antibody (CAB6876)
- SKU:
- CAB6876
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Signal Transduction
Frequently bought together:
Description
抗体名: | Anti-ATP6V1B1 Antibody |
抗体コード: | CAB6876 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB |
反応性: | Human, Mouse |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human ATP6V1B1 (NP_001683.2). |
申し込み: | WB |
推奨希釈: | WB 1:500 - 1:2000 |
反応性: | Human, Mouse |
ポジティブサンプル: | BT-474, MCF7, HepG2, Mouse testis, Mouse kidney |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human ATP6V1B1 (NP_001683.2). |
精製方法: | Affinity purification |
ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
アイソタイプ: | IgG |
順序: | 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 |
遺伝子ID: | 525 |
Uniprot: | P15313 |
セルラーロケーション: | Endomembrane system, Peripheral membrane protein |
計算された分子量: | 56kDa |
観察された分子量: | 57kDa |
同義語: | ATP6V1B1, ATP6B1, RTA1B, VATB, VMA2, VPP3 |
バックグラウンド: | 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 |
View AllClose