FKRP
FKRP蛋白,即Fukutin相关蛋白,是一种关键的高尔基体内蛋白质,在转录酶转化过程中扮演重要角色,对于维持细胞正常功能至关重要。其基因突变与多种常染色体隐性肌肉病相关,包括从婴儿早期发病到相对缓慢进展的多种表型。其中,FKRP相关的肢带肌营养不良R9型是最常见的表型,也是先天性1C型肌营养不良症、肌眼脑病和Walker-Warburg综合征的致病基因,这些疾病表现为更严重的表型。FKRP蛋白不仅是先天性肌营养不良症等严重疾病的致病基因,还涉及到其他生物过程。
热销产品
验证数据
IHC image of CSB-PA862050LA01HU diluted at 1:400 and staining in paraffin-embedded human ovarian cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
IHC image of CSB-PA862050LA01HU diluted at 1:400 and staining in paraffin-embedded human placenta tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
FKRP Antibodies
FKRP for Homo sapiens (Human)
产品货号 | 产品名称 | 种属反应性 | 应用类型 |
---|---|---|---|
CSB-PA035204 | FKRP Antibody | Human,Mouse,Rat | ELISA,WB |
CSB-PA060135 | FKRP Antibody | Human,Mouse,Rat | WB, IF, ELISA |
CSB-PA862050LA01HU | FKRP Antibody | Human | ELISA, IHC |
CSB-PA862050LB01HU | FKRP Antibody, HRP conjugated | Human | ELISA |
CSB-PA862050LC01HU | FKRP Antibody, FITC conjugated | Human | |
CSB-PA862050LD01HU | FKRP Antibody, Biotin conjugated | Human | ELISA |
FKRP Proteins
FKRP Proteins for Homo sapiens (Human)
产品货号 | 产品名称 | 来源 |
---|---|---|
CSB-YP862050HU CSB-EP862050HU CSB-BP862050HU CSB-MP862050HU CSB-EP862050HU-B |
Recombinant Human Fukutin-related protein (FKRP), partial | Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |