Overview
| 别名 | Presequence protease; mitochondrial; hPreP; Pitrilysin metalloproteinase 1; Metalloprotease 1; hMP1 |
| 基因名 | PITRM1 |
| UniProt ID | Q5JRX3 |
| 反应种属 | Human |
| 应用 | WB,IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 1P2-R7-J8 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 117 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000; IHC-1:100-1:200 |
| 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 | Pitrilysin metallopeptidase 1 (PITRM1), also known as presequence protease (PreP), is a 117 kDa ATP-dependent zinc metallopeptidase located in the mitochondrial matrix, where it plays a crucial role in mitochondrial proteostasis by degrading post-cleavage mitochondrial targeting sequences and a broad range of unstructured peptides, including amyloid beta (Aβ) fragments. Structurally, PITRM1 consists of two homologous domains (hPreP-N and hPreP-C) connected by a hinge region, forming a large catalytic chamber that enables size-exclusion-based substrate selection, allowing degradation of peptides between 10 and 65 amino acids but not larger folded proteins. The enzyme is essential for mitochondrial function, as it prevents the accumulation of toxic peptides that could impair mitochondrial activity. Clinically, PITRM1 deficiency has been linked to neurodegenerative diseases such as Alzheimer's disease, due to impaired degradation of mitochondrial Aβ, and is associated with conditions like spinocerebellar ataxia, autosomal recessive 30, and mitochondrial DNA depletion syndrome 7. |
检测原理