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Niemann Pick C1 Antibody
Niemann Pick C1 Antibody
- 中文名称：
- Niemann Pick C1 Antibody
- 英文名称：
- Niemann Pick C1 Antibody
- 品牌：
- AAA Biotech
- 品牌介绍：
- AAA Biotech专注于为全球生命科学研究提供高品质的蛋白质研究工具，核心产品包括经严格验证的抗体、重组蛋白及ELISA试剂盒。
- 货号：
- AAA30458
- 规格：
- 0.05 mL|0.1 mL|2x0.1 mL|3x0.1 mL|4x0.1 mL
- 保存建议：
- 储存在-20摄氏度
- 货期：
- 6-8周
- 来源宿主：
- Rabbit
- 反应种属：
- Human, Mouse, Rat
- 应用：
- WB (Western Blot), ICC (Immunocytochemistry), IF (Immunofluorescence), IHC (Immunohistochemistry), FCM/FACS (Flow Cytometry)
- 免责声明：
- *本产品仅供科研实验使用，不得用于临床诊断。*
- 其他：
克隆性:Monoclonal
同型:N/A
克隆号:[JB87-33]
特异性:N/A
纯度:ProA affinity purified
形式：1*TBS (pH7.4), 1% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
浓度：N/A
- 说明书：
Cells obtain cholesterol via two distinct pathways, endogenous synthesis in the endoplasmic reticulum and exogenous uptake through the low-density lipoprotein (LDL) receptor pathway. NPC1 is a protein that resides in late endosomes and lysosomes and is involved in the intracellular trafficking of cholesterol. The human NPC1 gene maps to chromosome 18q11.2 and produces proteins which undergo N-glycosylation and are expressed in brain and liver. NPC1 contains a cysteine-rich domain, which is critical for proper protein function, but is highly mutated. Mutations in NPC1 result in Niemann-Pick disease type C (NPC), an autosomal recessive disease characterized by the accumulation of unesterified cholesterol in the endosomal/lysosomal system. The accumulation of cholesterol results in progressive neurodegeneration and death. More than 90% of cases of NPC are due to mutations in NPC1 and patients with NPC display multiple neurological symptoms, such as hepatosplenomegaly, ataxia, dystonia and dementia.
