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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

FKRP Antibody

驗證數(shù)據(jù)

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)