Your Good Partner in Biology Research

中文简体

CSF2｜ CTLA4｜ DLL3｜ EGFR｜ IL4｜ IL4R｜ LAG3｜ MSTN｜ NECTIN4｜ NT5E｜ SEZ6｜ TM4SF1｜ TNFSF13｜ TPO

  1. 首页
  2. 蛋白
  3. 重组蛋白
  4. Recombinant Mouse Niemann-Pick C1 protein (Npc1), partial

Recombinant Mouse Niemann-Pick C1 protein (Npc1), partial

  • 中文名称：
    小鼠Npc1重组蛋白
  • 货号：
    CSB-YP015974MO
  • 规格：
  • 来源：
    Yeast
  • 其他：
  • 中文名称：
    小鼠Npc1重组蛋白
  • 货号：
    CSB-EP015974MO
  • 规格：
  • 来源：
    E.coli
  • 其他：
  • 中文名称：
    小鼠Npc1重组蛋白
  • 货号：
    CSB-EP015974MO-B
  • 规格：
  • 来源：
    E.coli
  • 共轭：
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他：
  • 中文名称：
    小鼠Npc1重组蛋白
  • 货号：
    CSB-BP015974MO
  • 规格：
  • 来源：
    Baculovirus
  • 其他：
  • 中文名称：
    小鼠Npc1重组蛋白
  • 货号：
    CSB-MP015974MO
  • 规格：
  • 来源：
    Mammalian cell
  • 其他：

产品详情

  • 纯度：
    >85% (SDS-PAGE)
  • 基因名：
    Npc1
  • Uniprot No.：
    O35604
  • 别名：
    Npc1; NPC intracellular cholesterol transporter 1; Niemann-Pick C1 protein
  • 种属：
    Mus musculus (Mouse)
  • 蛋白长度：
    Partial
  • 蛋白标签：
    Tag type will be determined during the manufacturing process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 产品提供形式：
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 复溶：
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 货期：
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事项：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet ：
    Please contact us to get it.

产品评价

靶点详情

  • 功能：
    Intracellular cholesterol transporter which acts in concert with NPC2 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment. Unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes is transferred by NPC2 to the cholesterol-binding pocket in the N-terminal domain of NPC1. Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket. May play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals (Probable).
  • 基因功能参考文献：
    1. Mutation in the NPC1 gene is associated with Niemann-Pick type C. PMID: 28167839
    2. This study shown Microglia can aggravate olfactory dysfunction by mediating neuronal death in the olfactory bulb (OB) of a murine model of Niemann-Pick disease type C1 (NPC1) PMID: 27687148
    3. High-resolution respirometry analyses revealed that GSH-EE improved oxidative phosphorylation, coupled respiration and maximal electron transfer in cerebellum of Npc1(-/-) mice PMID: 27888692
    4. the spleen is significantly enlarged in Npc1(-/-) mice. PMID: 27860245
    5. Study is the first data to reveal motor and behavioral deficits in early maturity of Npc1+/- mice. PMID: 26942423
    6. Npc1 gene interacts with a high fat diet to promote weight gain through differential regulation of central energy metabolism pathways. PMID: 28487438
    7. AAV9-mediated NPC1 delivery significantly promoted Purkinje cell survival, restored locomotor activity and coordination, and increased the lifespan of NPC1(-/-) mice. Our work suggests that AAV-based gene therapy is a promising means to treat NPC disease. PMID: 28053186
    8. Male NPC1+/- mice had increased fat storage while eating a high-fat diet. PMID: 28130309
    9. Our data show that: i) HDAC2 levels and activity are increased in NPC neuronal models and in Npc1(-/-) mice; ii) inhibition of c-Abl or c-Abl deficiency prevents the increase of HDAC2 protein levels and activity in NPC neuronal models PMID: 26603102
    10. activation of the alternative pathway is involved in Niemann-Pick C1 associated pulmonary macrophage accumulation, with low proliferation of these cells balanced by low levels of apoptosis. PMID: 26707209
    11. This study showed that deleting the Npc1 gene is accompanied by an increase in germ cell apoptosis and compensatory imbalances in the expression of cholesterol enzymatic and transporter factors. PMID: 25009206
    12. These results show that NPC1 is critical for ebolavirus replication and pathogenesis in animals. PMID: 26015498
    13. There was a transient increase in biliary cholesterol concentration in Npc1(-/-) mice. PMID: 25115571
    14. data suggest that aberrant expression of Pcdhs is a pathological process accompanied by neurodegeneration in Npc1 mutant mice PMID: 24671883
    15. This study deministrated that lack of NPC1 in either neurons or glial cells did not affect the excitability of Purkinje cells, the formation of dendrites or their excitatory synaptic activity. PMID: 24910948
    16. Hearing loss is an early consequence of Npc1 gene deletion in the mouse model of Niemann-Pick disease, type C. PMID: 24839095
    17. These data show that cholesterol homeostasis through NPC1 plays a crucial role in maintaining insulin action at multiple levels in adipocytes. PMID: 24752197
    18. Data suggest that axonal pathologies in Npc1 mutant spinal cord are strongly correlated with the increase of activated glial cells, which produce IL-1beta and ApoE, resulting in activation of p38-MAPK signaling pathway and enhanced phosphorylated tau protein PMID: 24644136
    19. Metabolic profiling revealed markedly increased lactate and decreased acetate/acetyl-CoA levels in Npc1 (-/-) cerebellum and cerebral cortex at all ages PMID: 24367541
    20. NPC1(-/-) animals present myelin-like lysosomal deposits in virtually all types of cells of the peripheral and central olfactory system. PMID: 24391715
    21. Cav-1 has a regulatory role in pulmonary cholesterol metabolism when lysosomal sterol transport is disrupted in Npc1-deficient mice. PMID: 24747682
    22. Npc1 and npc2 deficiencies result in pulmonary abnormalities observed in human Niemann-Pick type C disease. PMID: 23843985
    23. NPC1 gene dosage effects in the metabolic features associated with type 2 diabetes mellitus. PMID: 23769925
    24. lipid uptake and intracellular transport by neurons and oligodendrocytes through an Npc1-dependent pathway is required for both the formation and maintenance of CNS myelin. PMID: 23593041
    25. Results demonstrate that HP-beta-CD works in cells of Npc1(-/-) mice by rapidly liberating lysosomal cholesterol for normal sterol processing within the cytosolic compartment. PMID: 22892156
    26. These results suggest a novel mechanism for the interaction of fatty acids with the Npc1 gene to influence energy balance and to promote weight gain. PMID: 22990467
    27. The results of this study suggest that at least in the somatosensory neocortex NPC1 protein is instrumental in synaptic function. PMID: 22325094
    28. Data found an increase in ceruloplasmin levels in the plasma of Npc1 -/- mice compared to Npc1 +/+ mice, and this increase was statistically significant (*p < 0.05). PMID: 22526561
    29. Npc1(-/-) mice that sensory thalamic pathways are particularly vulnerable to loss of NPC1 resulting in neurodegeneration in Npc1(-/-) mice. PMID: 22198570
    30. Data show that global deletion of Npc1 in adult mice leads to progressive weight loss, impaired motor function and early death in a time course similar to that resulting from germline deletion. PMID: 21856732
    31. NPC1 and NPC2 proteins participate in endosomal/lysosomal processing of both sphingolipids and cholesterol PMID: 21708114
    32. In liver, absence of either NPC1 or NPC2 resulted in similar alterations in the carbohydrate processing of the lysosomal protease, tripeptidyl peptidase I. PMID: 21887293
    33. Npc1 gene interacts with both a high-fat diet and modifying genes to promote additive weight gain, consistent with common human obesity being a complex multifactorial disease. PMID: 21815257
    34. The NPC1 gene now represents a previously unrecognized gene involved in maintaining energy and metabolic homeostasis. PMID: 21036943
    35. Npc1 deficiency in the C57BL6/J background is associated with spleen, erythrocyte, and immune system abnormalities that lead to a reduced lifespan. PMID: 21910975
    36. The cholesterol binding site in the N-terminal domain of NPC1 is essential for cholesterol export from lysosomes in living animals as it is in cultured cells. PMID: 21896731
    37. Weekly cyclodextrin administration overcomes the lysosomal transport defect associated with the NPC1 mutation, nearly normalizes hepatic and whole animal cholesterol pools, and prevents the development of liver disease. PMID: 20581737
    38. Hepatic NPC1 expression is an important factor for regulating the biliary secretion of diet-derived cholesterol as well as for diet-induced cholesterol gallstone formation in mice. PMID: 20408952
    39. decreased Npc1 gene dosage resulting in decreased Npc1 protein function, promoted weight gain in mice fed a high-fat diet consistent with a gene-diet interaction PMID: 19910939
    40. In mice, loss of NPC1 function reduces hepatocyte TG content and secretion by increasing the metabolic flux of carbons into cholesterol synthesis. PMID: 20489167
    41. Expression of genes in six pathways was significantly altered in the cerebellums of Npc1-/- mice; results provide novel molecular insight regarding the mechanisms of pathogenesis in NPC disease and reveal potential new therapeutic targets PMID: 20153740
    42. a transport pathway for endosomal cholesterol to mitochondria that requires MLN64, but not NPC1 PMID: 19965586
    43. NPC1 deficiency over the period examined caused drastic defects on exocytosis in both glutamatergic and GABAergic synapses. PMID: 20167265
    44. Npc1 deficiency leads to cell autonomous, selective neurodegeneration and suggest that the ataxic symptoms of NPC disease arise from Purkinje cell death rather than cellular dysfunction PMID: 20007718
    45. Data report that Niemann-Pick type C1 (Npc1) is distributed in distinct layers of the mouse retina and that its deficiency causes striking retinal degeneration in 2-month-old mice with signs of age-related maculopathies. PMID: 19883762
    46. correction of sterility in GFAP-Npc1E, Npc1(-/-) mice is a result of restoring hypothalamic control of the pituitary PMID: 19875890
    47. Data demonstrate that the cellular PrP(Sc) content decreases upon accumulation of cholesterol in late endosomes, as induced by NPC-1 knock-down. PMID: 19823766
    48. mdr1a deficiency corrects sterility in Niemann-Pick C1 protein deficient female mice PMID: 11984826
    49. NPC1, the gene for Niemann-Pick type C disease, characterized by an accumulation of cholesterol in most tissues, promoted B. abortus infection. PMID: 12183525
    50. involved in enhanced ABCA1 degradation in ABCA1-mediated cholesterol efflux which is defective in free cholesterol-loaded macrophages PMID: 12215451

    显示更多

    收起更多

  • 亚细胞定位：
    Late endosome membrane; Multi-pass membrane protein. Lysosome membrane; Multi-pass membrane protein.
  • 蛋白家族：
    Patched family
  • 组织特异性：
    Detected in liver (at protein level). Ubiquitous. Detected in adult heart, spleen, lung, liver, skeletal muscle, kidney, testis.
  • 数据库链接：

    KEGG: mmu:18145

    STRING: 10090.ENSMUSP00000025279

    UniGene: Mm.3484

Most popular with customers

产品中心
特别关注


X
九游平台-九游(中国) | 米兰体育-米兰体育（中国） | 开云手机在线登陆入口 | 江南官方体育网页版 | 开云网页版 | 开云官方在线入口 | 开云电子体育 | 开云官方链接 | 江南网页版页面登录 |