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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

FKRP Antibody

验证数据

IHC
CSB-EP017315HUb3 IHC-1

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
CSB-EP017315HUb3 IHC-2

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)

FKRP Proteins

FKRP Proteins for Homo sapiens (Human)