FKRP protein, also known as Fukutin-related protein, is a key protein in the Golgi apparatus that plays an important role in the conversion process of transcription enzymes and is crucial for maintaining normal cellular functions. Its gene mutations are associated with various autosomal recessive muscle diseases, including various phenotypes ranging from early onset in infancy to relatively slow progression. Among them, FKRP-related limb-girdle muscular dystrophy type R9 is the most common phenotype and is also a pathogenic gene for congenital type 1C muscular dystrophy, myo-ocular-encephalopathy, and Walker-Warburg syndrome, which exhibit more severe phenotypes. FKRP protein is not only a pathogenic gene for severe diseases such as congenital muscular dystrophy, but also involved in other biological processes.
Validated Data
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.
The following FKRP reagents supplied by CUSABIO are manufactured under a strict quality control system. Multiple applications have been validated and solid technical support is offered.
FKRP Antibodies for Homo sapiens (Human)
Code | Product Name | Species Reactivity | Application |
---|---|---|---|
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 for Homo sapiens (Human)
Code | Product Name | Source |
---|---|---|
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 |