Anti-ATP6V0A4 Antibody (CAB7591)
- SKU:
- CAB7591
- 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-ATP6V0A4 Antibody |
| 抗体コード: | CAB7591 |
| 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 ATP6V0A4 (NP_065683.2). |
| 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: | A375, Mouse kidney |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human ATP6V0A4 (NP_065683.2). |
| 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: | 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 |
| 遺伝子ID: | 50617 |
| Uniprot: | Q9HBG4 |
| セルラーロケーション: | Apical cell membrane, Multi-pass membrane protein |
| 計算された分子量: | 96kDa |
| 観察された分子量: | 70-90kDa |
| 同義語: | ATP6V0A4, A4, ATP6N1B, ATP6N2, RDRTA2, RTA1C, RTADR, STV1, VPH1, VPP2 |
| 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 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 |
 | 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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