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NPC2 Antibody

  • 货号：
    CSB-PA015976LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA015976LA01HU at dilution of 1:100
    • Immunofluorescent analysis of A549 cells using CSB-PA015976LA01HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) NPC2 Polyclonal antibody
  • Uniprot No.：
    P61916
  • 基因名：
  • 别名：
    EDDM1 antibody; Epididymal protein 1 antibody; Epididymal secretory protein antibody; Epididymal secretory protein E1 antibody; hE1 antibody; Human epididymis-specific protein 1 antibody; Niemann-Pick disease type C2 antibody; Niemann-Pick disease type C2 protein antibody; NPC intracellular cholesterol transporter 2 antibody; NPC2 antibody; NPC2_HUMAN antibody; Tissue specific secretory protein antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human NPC intracellular cholesterol transporter 2 protein (20-151AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，NPC2 Antibody (CSB-PA015976LA01HU)，的标记方式是Non-conjugated。对于NPC2 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA015976LB01HU NPC2 Antibody, HRP conjugated ELISA
    FITC CSB-PA015976LC01HU NPC2 Antibody, FITC conjugated
    Biotin CSB-PA015976LD01HU NPC2 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC, IF
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:20-1:200
    IF 1:50-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    Intracellular cholesterol transporter which acts in concert with NPC1 and plays an important role in the egress of cholesterol from the 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. May bind and mobilize cholesterol that is associated with membranes. NPC2 binds cholesterol with a 1:1 stoichiometry. Can bind a variety of sterols, including lathosterol, desmosterol and the plant sterols stigmasterol and beta-sitosterol. The secreted form of NCP2 regulates biliary cholesterol secretion via stimulation of ABCG5/ABCG8-mediated cholesterol transport.
  • 基因功能参考文献：
    1. Results propose that, depending on the location of the cholesterol ligand, a dynamical interface between the NPC2 and NPC1 N-terminal domain (NTD) proteins exists. Structural features of a particular interface can lower the energy barrier and stabilize the passage of the cholesterol substrate from NPC2 to NPC1(NTD). PMID: 30181526
    2. Our study demonstrated that NPC2-mediated free cholesterol homeostasis controls hepatic stellate cells proliferation and mitochondrial function. PMID: 29874879
    3. Niemann-Pick disease type C .E118X NPC2 gene mutation may be prevalent among individuals in Anatolia. PMID: 28808920
    4. The heterozygous mutations of NPC2 gene could contribute to dementia plus, at least in a subset of patients. PMID: 27792009
    5. Stopped-Flow Fluorescence Methods for Investigating Intracellular Cholesterol Transport Mechanisms of NPC2 Protein. PMID: 28205170
    6. Docking of the NPC1-NPC2 complex onto the full-length NPC1 structure reveals a direct cholesterol transfer tunnel between NPC2 and N-terminal domain cholesterol binding pockets, supporting the "hydrophobic hand-off" cholesterol transfer model. PMID: 27551080
    7. identification of NPC1 and/or NPC2 mutations combined with descriptions of clinical phenotype, will improve our knowledge of pathogenic mutations and our understanding of genotype-phenotype correlations. PMID: 27339554
    8. Overall, we provide a mechanism by which npc2-mediated cholesterol transport is controlled by the membrane composition and how npc2-lipid interactions can regulate the transport rate. PMID: 29084218
    9. Our results suggest that NPC2 is in a mitochondrially associated autophagosome and plays an important role in regulating mitophagy, mitochondrial quality control, and mitochondrial function. PMID: 27402802
    10. NEGR1 interacts with NPC2 and increases its protein stability PMID: 27940359
    11. suggest a general mechanism for NPC2 mediated sterol transfer, in which Phe66, Val96, and Tyr100 act as reversible gate keepers. These residues stabilize the sterol in the binding pose via pi-pi stacking but move transiently apart during sterol release PMID: 27533706
    12. Study demonstrates that Niemann-Pick type C disease can present in early years of life with pulmonary complications like alveolar proteinosis and hepatosplenomegaly or hepatomegaly due to mutation in NPC2 gene. PMID: 28095804
    13. Our data suggest an incidence rate for NPC1 and NPC2 of 1/92,104 and 1/2,858,998, respectively. Evaluation of common NPC1 variants, however, suggests that there may be a late-onset NPC1 phenotype with a markedly higher incidence. PMID: 25764212
    14. Structure of glycosylated NPC1 luminal domain C reveals insights into NPC2 and Ebola virus interactions PMID: 26846330
    15. hypothesize that, in part, NPC2 rapidly traffics cholesterol between closely appositioned membranes within the multilamellar interior of late endosomal/lysosomal proteins, ultimately effecting cholesterol egress from this compartment PMID: 26296895
    16. NPC2 may play an important role in negatively regulate cell proliferation. PMID: 25754535
    17. heterozygous mutations in the NPC1/2 gene might be a risk factor for Alzheimer's disease PMID: 25220527
    18. Data suggest that in order for the ligand cholesterol to slide from one binding pocket to the other (from NPC2 to NPC1), cholesterol undergoes conformational change/isomerization to accommodate the bent transfer pathway between the 2 binding pockets. PMID: 25251378
    19. Suggest role for mouse epididymal NPC2 in regulating male fertility. PMID: 24709320
    20. twelve individuals were subsequently confirmed to be NP-C by DNA analysis of NPC1 and NPC2 genes, with the early infantile form, the late infantile form, the juvenile form, and the adult form PMID: 24915861
    21. Recombinant NPC2 protein increases triglyceride levels in body fat. PMID: 24438076
    22. these results suggest that the vitamin A-mediated antimicrobial mechanism against M. tuberculosis requires NPC2-dependent expression and function, indicating a key role for cellular cholesterol regulation in the innate immune response. PMID: 24501203
    23. The p.Pro120Ser causing mutation in NPC2 observed in the Iranian patients was earlier observed in the only other NPC2 patient reported from the Middle East. PMID: 23791309
    24. an atomistic model is proposed of the transfer of cholesterol from NPC2 to NPC1(NTD) through the formation of an intermediate NPC1(NTD)-NPC2 complex PMID: 24001314
    25. NPC2-deficiency leads to a dramatic up-regulation of the arachidonic acid (AA) metabolic pathway in human fibroblasts. PMID: 23814065
    26. Treatment of NPC1-null or NPC2-deficient cells with cyclodextrin was effective in reducing cholesterol storage as well as the endocytic accumulation of sialoglycoproteins, demonstrating a direct link between cholesterol storage and abnormal recycling. PMID: 23733943
    27. The NPC2 delivers cholesterol to the perimeter membrane of late endosomes, where it becomes available for transport to mitochondria without requiring NPC1. PMID: 22962690
    28. overexpression of ABCA1 alone is able to correct the mobilization of cholesterol from late endosomes/lysosomes and the formation of HDL particles in NPC1- but not NPC2-deficient human fibroblasts PMID: 22179027
    29. This is the first report demonstrating that GNMT plays an important role in regulating cholesterol homeostasis via interaction with NPC2 PMID: 22183894
    30. Loss of Niemann Pick type C proteins 1 and 2 greatly enhances HIV infectivity and is associated with accumulation of HIV Gag and cholesterol in late endosomes/lysosomes. PMID: 22273177
    31. mechanism of sterol transport by cyclodextrins using in vitro model systems and fluorescence spectroscopy and NPC2-deficient fibroblasts PMID: 21740003
    32. NPC2 protein of certain cells forms papillae coupled with apoptosis that creates open space PMID: 21253586
    33. a novel mechanism where NPC2 by negatively regulating ERK 1/2 MAPK phosphorylation may efficiently suppress development of maladaptive tissue remodeling and inflammation. PMID: 21084287
    34. NPC2 protein interacts N-ternimal domain of NPC1 protein, thereby opening an entry pore on NPC1 protein and allowing cholesterol to transfer without passing through the water phase. PMID: 20674861
    35. NPC2 as a novel intracrine/autocrine factor that controls adipocyte differentiation and function PMID: 20650896
    36. physiological and coordinate downregulation of the NPC1 and NPC2 genes/proteins promotes the sequestration of LDL-derived cholesterol within endocytic compartments and serves a role in maintaining intracellular cholesterol homeostasis PMID: 19746448
    37. The results suggest that NPC1 and NPC2 can function independently of one another in the egress of certain membrane-impermeable lysosomal cargo. PMID: 20007703
    38. Review of NPC1 and HE1/NPC2 roles regulating cholesterol transport through endosomal/lysosomal system and in Niemann-Pick type C disease PMID: 12125814
    39. NPC2, NPC1 and MLN64 may act in an ordered sequence to sense cholesterol, effect sterol movement, and consequently, influence the process of vesicular trafficking. PMID: 12398991
    40. Adult-onset NPC2 with lysosomal storage virtually restricted to neurons represents a novel phenotypic and genotypic variant with diffuse cognitive impairment and focal frontal involvement described for the first time. PMID: 12447927
    41. NPC2 protein has binding sites with a role in efficient secretion PMID: 12591949
    42. NPC1 and NPC2 have a role in the regulation of sterol homeostasis through generation of LDL cholesterol-derived oxysterols PMID: 12719428
    43. The HE1 transcript was affected by the obstruction of the epididymis with little or no mRNA detectable along the epididymis. PMID: 14662784
    44. Functional characterization of the mutant proteins showed an excellent genotype-phenotype correlation in the three cases for whom a clinical history was available PMID: 15937921
    45. NPC2 is secreted from the liver into bile and plasma, where it may have a functional role in cholesterol transport in normal and disease conditions. PMID: 16374838
    46. NPC2 protein has a role in the egress of LDL derived cholesterol out of the endosomal/lysosomal compartment PMID: 16606609
    47. Compared with normal men, seminal plasma of vasectomized men is characterized by a major decrease in immunodetectable HE1/NPC2 and surgical vasectomy reversal will normalize seminal plasma HE1/NPC2 amount to similar level of that of normal mem. PMID: 16772431
    48. NPC2 has a direct and specialized function in lysosomal sterol transport PMID: 17018531
    49. p.P120S mutation, the first naturally occurring missense mutation located in the cholesterol-binding Evolutionarily Constrained Regions D domain, results in reduced amounts of a protein capable to reach the lysosome, but unable to bind cholesterol. PMID: 17470133
    50. role of the AP-3 pathway in targeting of NP-C proteins; study found although mouse NPC1 & hNPC2 co-localize with AP-3 to a similar extent in fibroblasts, hNPC2 preferentially co-localizes with AP-1; targeting of both NPC1 & NPC2 is dependent on AP-3 PMID: 17895371

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  • 相关疾病：
    Niemann-Pick disease C2 (NPC2)
  • 亚细胞定位：
    Secreted. Endoplasmic reticulum. Lysosome.
  • 蛋白家族：
    NPC2 family
  • 组织特异性：
    Detected in gallbladder bile. Detected in fibroblasts, kidney, liver, spleen, small intestine, placenta and testis (at protein level). Epididymis.
  • 数据库链接：

    HGNC: 14537

    OMIM: 601015

    KEGG: hsa:10577

    STRING: 9606.ENSP00000451112

    UniGene: Hs.433222