Overview
| 别名 | Transitional endoplasmic reticulum ATPase; TER ATPase; 15S Mg(2+)-ATPase p97 subunit; Valosin-containing protein; VCP |
| 基因名 | VCP |
| UniProt ID | P55072 |
| 反应种属 | Human,Mouse |
| 应用 | WB,IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 1A7-R8-Y6 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 89 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000; IHC-1:10000 |
| 存储缓冲液 | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.09% sodium azide |
| 保存温度 | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| 背景信息 | Valosin-containing protein (VCP), also known as p97, is a hexameric AAA+ ATPase essential for cellular homeostasis, particularly in proteostasis. Comprising 806 amino acids with a molecular weight of about 97 kDa, VCP features an N-terminal domain for substrate and cofactor binding, two ATPase domains (D1 and D2) that hydrolyze ATP to drive hexamer assembly and unfold proteins via a central pore, and a C-terminal domain for partner interactions. It governs diverse processes like ubiquitin-proteasome degradation, ER-associated degradation (ERAD), autophagy, mitophagy, lysophagy, stress granule dynamics, DNA repair, and mitosis by collaborating with over 30 cofactors such as UFD1L and PLAA. VCP mutations, often heterozygous missense variants like R155H or R93C, dominantly cause multisystem proteinopathy 1 (MSP1), manifesting as inclusion body myopathy, Paget’s disease of bone, frontotemporal dementia, amyotrophic lateral sclerosis, and sometimes sensory or cardiac involvement. These impair ATPase activity, autophagic flux, and lysosomal clearance, leading to aggregate accumulation and neurodegeneration. |
检测原理