分享产品介绍 评论(0)
宿主来源
Rabbit抗原名称
BSND分子别名
Barttin; BART免疫原
Synthetic Peptide细胞定位
Cell membraneAccession
Q8WZ55克隆号
S-3815-70抗体类型
Recombinant mAb抗体同种型
IgG反应种属 ?
Hu, Ms, Rt纯化方式
Protein A浓度
0.5 mg/ml标记
Unconjugated性状
Liquid缓冲体系
PBS, 40% Glycerol, 0.05% BSA, 0.03% Proclin 300
储存条件
12 months from date of receipt / reconstitution, -20 °C as supplied
应用
IHC-P ?
IF ?
稀释度
应用 稀释度 推荐种属 IHC-P 1:2000 Hu, Ms, Rt IF 1:500 Hu
BSND (Barttin CLCNK Type Beta Subunit) is a critical auxiliary subunit encoded by the BSND gene that is essential for the proper functioning of ClC-Ka and ClC-Kb chloride channels, which are predominantly expressed in the kidney's thick ascending limb of the loop of Henle and the stria vascularis of the inner ear. Unlike typical channel subunits that merely modulate gating, Barttin acts as an obligatory beta-subunit required for the trafficking of these alpha-subunits from the endoplasmic reticulum to the plasma membrane, thereby enabling chloride ion transport necessary for salt reabsorption and endolymph production. Loss-of-function mutations in the BSND gene disrupt this complex assembly, leading to impaired renal sodium-potassium-chloride cotransport and defective potassium recycling in the cochlea, which clinically manifests as autosomal recessive type IV Bartter syndrome characterized by severe antenatal polyhydramnios, profound salt-wasting nephropathy, metabolic alkalosis, and congenital sensorineural deafness, distinguishing it from other forms of Bartter syndrome that typically preserve hearing.
免疫组化
IHC shows positive staining in paraffin-embedded human kidney. Anti-BSND antibody was used at 1/2000 dilution, followed by a HRP Polymer for Rabbit IgG (ready to use). Counterstained with hematoxylin. Heat mediated antigen retrieval with Tris/EDTA buffer pH9.0 was performed before commencing with IHC staining protocol.
IHC shows positive staining in paraffin-embedded human renal oncocytoma. Anti-BSND antibody was used at 1/2000 dilution, followed by a HRP Polymer for Rabbit IgG (ready to use). Counterstained with hematoxylin. Heat mediated antigen retrieval with Tris/EDTA buffer pH9.0 was performed before commencing with IHC staining protocol.
IHC shows positive staining in paraffin-embedded human chromophobe renal cell carcinoma. Anti-BSND antibody was used at 1/2000 dilution, followed by a HRP Polymer for Rabbit IgG (ready to use). Counterstained with hematoxylin. Heat mediated antigen retrieval with Tris/EDTA buffer pH9.0 was performed before commencing with IHC staining protocol.
Negative control: IHC shows negative staining in paraffin-embedded human renal clear cell carcinoma. Anti-BSND antibody was used at 1/2000 dilution, followed by a HRP Polymer for Rabbit IgG (ready to use). Counterstained with hematoxylin. Heat mediated antigen retrieval with Tris/EDTA buffer pH9.0 was performed before commencing with IHC staining protocol.
Negative control: IHC shows negative staining in paraffin-embedded human papillary renal cell carcinoma. Anti-BSND antibody was used at 1/2000 dilution, followed by a HRP Polymer for Rabbit IgG (ready to use). Counterstained with hematoxylin. Heat mediated antigen retrieval with Tris/EDTA buffer pH9.0 was performed before commencing with IHC staining protocol.
IHC shows positive staining in paraffin-embedded mouse kidney. Anti-BSND antibody was used at 1/2000 dilution, followed by a HRP Polymer for Rabbit IgG (ready to use). Counterstained with hematoxylin. Heat mediated antigen retrieval with Tris/EDTA buffer pH9.0 was performed before commencing with IHC staining protocol.
IHC shows positive staining in paraffin-embedded rat kidney. Anti-BSND antibody was used at 1/2000 dilution, followed by a HRP Polymer for Rabbit IgG (ready to use). Counterstained with hematoxylin. Heat mediated antigen retrieval with Tris/EDTA buffer pH9.0 was performed before commencing with IHC staining protocol.
免疫荧光
IF shows positive staining in paraffin-embedded Human kidney. Anti-BSND antibody was used at 1/500 dilution (Green) and incubated overnight at 4°C. Goat polyclonal Antibody to Rabbit IgG - H&L (Alexa Fluor® 488) was used as secondary antibody at 1/1000 dilution. Counterstained with DAPI (Blue). Heat mediated antigen retrieval with EDTA buffer pH9.0 was performed before commencing with IF staining protocol.
组织表达图谱
Expression of BSND in human tissues.
Expression of BSND in mouse & rat tissues.
评论(0)