FKRP
FKRP蛋白，即Fukutin相關(guān)蛋白，是一種關(guān)鍵的高爾基體內(nèi)蛋白質(zhì)，在轉(zhuǎn)錄酶轉(zhuǎn)化過程中扮演重要角色，對于維持細(xì)胞正常功能至關(guān)重要。其基因突變與多種常染色體隱性肌肉病相關(guān)，包括從嬰兒早期發(fā)病到相對緩慢進(jìn)展的多種表型。其中，F(xiàn)KRP相關(guān)的肢帶肌營養(yǎng)不良R9型是最常見的表型，也是先天性1C型肌營養(yǎng)不良癥、肌眼腦病和Walker-Warburg綜合征的致病基因，這些疾病表現(xiàn)為更嚴(yán)重的表型。FKRP蛋白不僅是先天性肌營養(yǎng)不良癥等嚴(yán)重疾病的致病基因，還涉及到其他生物過程。
熱銷產(chǎn)品
驗證數(shù)據(jù)
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)
| 產(chǎn)品貨號 | 產(chǎn)品名稱 | 種屬反應(yīng)性 | 應(yīng)用類型 |
|---|---|---|---|
| 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)
| 產(chǎn)品貨號 | 產(chǎn)品名稱 | 來源 |
|---|---|---|
| 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 |
