Overview
| 别名 | Mannosyl-oligosaccharide 1; 2-alpha-mannosidase IA; Man(9)-alpha-mannosidase; Man9-mannosidase; Mannosidase alpha class 1A member 1; Processing alpha-1; 2-mannosidase IA; Alpha-1; 2-mannosidase IA |
| 基因名 | MAN1A1 |
| UniProt ID | P33908 |
| 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 | 2D3-Z2-O9 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 72 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 | Mannosidase alpha class 1A member 1 (MAN1A1) is a Golgi-resident, single-pass type II transmembrane enzyme belonging to glycosyl hydrolase family 47. It catalyzes the progressive trimming of alpha-1,2-linked mannose residues from Man9GlcNAc2 to Man5GlcNAc2 on Asn-linked oligosaccharides, representing a critical step in glycoprotein maturation and quality control. By facilitating N-linked glycan processing, MAN1A1 ensures proper protein folding and trafficking within the secretory pathway. The enzyme participates in vesicle-mediated transport and subsequent modification of glycoproteins in the Golgi apparatus. Pathogenic variants in MAN1A1 are associated with autosomal recessive Rafiq syndrome, a neurodevelopmental disorder characterized by intellectual disability and speech delay, as well as congenital disorder of glycosylation type In. Disruption of MAN1A1 function leads to defective N-glycan processing and impaired ER-Golgi trafficking, contributing to multisystem pathology. |
检测原理