Overview
| 别名 | Tectonin beta-propeller repeat-containing protein 1 |
| 基因名 | TECPR1 |
| UniProt ID | Q7Z6L1 |
| 反应种属 | Human |
| 应用 | IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG2b |
| 克隆号 | 6Q2-G2-N4 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 129 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | IHC-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 | Tectonin beta-propeller repeat containing 1 (TECPR1) is an essential tethering factor that mediates the fusion of autophagosomes with lysosomes. It localizes to lysosomal membranes and specifically binds the ATG12–ATG5 conjugate and phosphatidylinositol-3-phosphate (PtdIns3P) to facilitate autophagic maturation. This process is critical for the selective degradation of protein aggregates, damaged mitochondria, and intracellular pathogens. Depletion of TECPR1 leads to the accumulation of autophagosomes and the buildup of p62 and LC3-II, indicating impaired autophagic flux. Clinically, mutations in TECPR1 are the primary cause of Hereditary Spastic Paraplegia 49 (SPG49), a neurodegenerative disorder characterized by early-onset spasticity and intellectual disability. It is also associated with Frontotemporal Dementia and Amyotrophic Lateral Sclerosis 7 (FTDALS7), where impaired protein clearance leads to motor neuron loss. Enhancing TECPR1 activity has been explored as a strategy to bolster autophagic flux in neurodegenerative models. |
检测原理