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LMAN1 Antibody

  • 中文名称：
    LMAN1兔多克隆抗体
  • 货号：
    CSB-PA012991LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western Blot
      Positive WB detected in: HepG2 whole cell lysate, Mouse heart tissue
      All lanes: LMAN1 antibody at 5.3µg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 58 kDa
      Observed band size: 58 kDa
    • IHC image of CSB-PA012991LA01HU diluted at 1:300 and staining in paraffin-embedded human adrenal gland tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • Immunofluorescence staining of A549 cells with CSB-PA012991LA01HU at 1:100, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was 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) LMAN1 Polyclonal antibody
  • Uniprot No.：
    P49257
  • 基因名：
  • 别名：
    LMAN1; ERGIC53; F5F8D; Protein ERGIC-53; ER-Golgi intermediate compartment 53 kDa protein; Gp58; Intracellular mannose-specific lectin MR60; Lectin mannose-binding 1
  • 宿主：
    Rabbit
  • 反应种属：
    Human, Mouse
  • 免疫原：
    Recombinant Human Protein ERGIC-53 protein (154-329AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，LMAN1 Antibody (CSB-PA012991LA01HU)，的标记方式是Non-conjugated。对于LMAN1 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-PA012991LB01HU LMAN1 Antibody, HRP conjugated ELISA
    FITC CSB-PA012991LC01HU LMAN1 Antibody, FITC conjugated
    Biotin CSB-PA012991LD01HU LMAN1 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, WB, IHC, IF
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:200-1:500
    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：
    Mannose-specific lectin. May recognize sugar residues of glycoproteins, glycolipids, or glycosylphosphatidyl inositol anchors and may be involved in the sorting or recycling of proteins, lipids, or both. The LMAN1-MCFD2 complex forms a specific cargo receptor for the ER-to-Golgi transport of selected proteins.
  • 基因功能参考文献：
    1. Combined deficiency of factors V and VIII by chance coinheritance of parahaemophilia and haemophilia A, but not by mutations of either LMAN1 or MCFD2 PMID: 29082580
    2. Genetic variants in the exon1 of MBL gene per se are not risk factors for Systemic lupus erythematosus (SLE) in South Indian Tamils. However, the association of codon 54 (rs1800450) with medium vessel vasculitis suggests that it may be a genetic modifier of clinical phenotype in SLE. PMID: 28097447
    3. Mannan-binding lectin reduces CpG DNA-induced inflammatory cytokine production in monocytes. PMID: 25664598
    4. MMP-9 secretion was reduced in the LMAN1 knockout cell line compared to control cells confirming the functional role of LMAN1. PMID: 26150355
    5. s identified a class of pathogen-derived ERGIC-53 ligands, a lectin-independent basis for their association with ERGIC-53, and a role for ERGIC-53 in the propagation of several highly pathogenic RNA virus families. PMID: 24237698
    6. Studies indicate that the LMAN1-CRD contains distinct, separable binding sites for both its partner protein MCFD2 and the cargo proteins FV/FVIII. PMID: 23852824
    7. Data indicate that together with its soluble coreceptor MCFD2, LMAN1 transports coagulation factors V (FV) and VIII (FVIII). PMID: 23709226
    8. Mutations in LMAN1 lead to F5F8D (combined deficiency of factor V And factor VIII) due to alterations in LMAN1-MCFD2 complex of coat protein (COP)II complex trafficking machinery; 70% of F5F8D patients have mutations in LMAN1. [REVIEW] PMID: 22764119
    9. UBXD1 modulates the trafficking of ERGIC-53-containing vesicles by controlling the interaction of transport factors with the cytoplasmic tail of ERGIC-53. PMID: 22337587
    10. Two new mutations at ERGIC-53 gene in a Turkish family. PMID: 20460353
    11. Data present the crystal structure of the LMAN1/MCFD2 complex and relate it to patient mutations. Circular dichroism data show that the majority of the substitution mutations give rise to a disordered or severely destabilized MCFD2 protein. PMID: 20138881
    12. Data show that mutations in MCFD2 that disrupt the tertiary structure and abolish LMAN1 binding still retain the FV/FVIII binding activities, suggesting that this interaction is independent of Ca(2+)-induced folding of the protein. PMID: 20007547
    13. Among Papua New Guinea malaria patients, 2 new mannose-binding lectin polymorphic promoter sites were found: one in the untranslated region at position +1 (G-->A, termed R/S), and the 2nd upstream of the gene at position -4 (G-->A, termed T/U). PMID: 12175909
    14. MBL deficiency is not a risk factor for SLE in women from the Canary Islands, but it is associated with lower prevalence of autoantibodies and with later age at disease onset and at SLE diagnosis. PMID: 12672193
    15. inactivating mutations in MCFD2 cause combined deficiency of factor V and factor VIII with a phenotype indistinguishable from that caused by mutations in LMAN1 PMID: 12717434
    16. Data show that the mRNA of lectin ERGIC-53 and its related protein VIP36 is induced by the known inducers of endoplasmic reticulum stress, tunicamycin and thapsigargin. PMID: 12727195
    17. an interaction between LMAN1 and FVIII in vivo was mediated via high mannose-containing asparagine-linked oligosaccharides that are densely situated within the B domain of FVIII, as well as protein-protein interactions PMID: 14629470
    18. Results describe the x-ray structure of human mannan-binding lectin-associated protein 19 (MAp19), and identify the residues involved in the interaction of MAp19 with mannan-binding lectin and L-ficolin. PMID: 15117939
    19. surfactant proteins A and D and mannose-binding lectin play roles in inflammation caused by DNA in lungs and other tissues PMID: 15145932
    20. ERGIC-53 and MCFD2 have important functions during cellular response to stress conditions PMID: 15292203
    21. The ERGIC-53 is stationary and not simply a collection of mobile carriers that mediate protein traffic from endoplasmic reticulum to Golgi. PMID: 15632110
    22. MBL gene polymorphism at codon 54 is not associated with the clearance of hepatitis B virus infection nor progression of disease in chronic hepatitis B virus infection PMID: 15716605
    23. MBL1 contains 9 disulfide-linked chains and is therefore trimeric in structure; the oligomerization state of MBL has a direct effect on its carbohydrate-binding properties, but no influence on the interaction with MBL-associated serine proteases (MASPs). PMID: 15728497
    24. LMAN1 and MCFD2 form a cargo receptor complex and the primary sorting signals residing in the B domain direct the binding of factor VIII PMID: 15886209
    25. Mutations in (LMAN1) and (MCFD2), have been found to be responsible for the dual deficiency of FV and FVIII. PMID: 16044454
    26. ERGIC-53 accumulated at the perinuclear region, and remained there even after cells were treated with agents that induce redistribution of Golgi proteins to the ER, indicating an inhibition of Golgi-to-ER transport of ERGIC-53. PMID: 16054885
    27. role in Aspergillus mediated allergies and infections PMID: 16114131
    28. ERGIC-53 is present exclusively as a hexameric complex in cells PMID: 16257008
    29. Mannan-binding lectin activates C3 and the alternative complement pathway without involvement of C2 PMID: 16670774
    30. Our findings identify a novel ERGIC-53 substrate, and indicate that interactions with light chains or ERGIC-53 seed muDeltaCH1 condensation in different stations of the early secretory pathway. PMID: 16735443
    31. Here, we have observed that NCT N-linked oligosaccharides mediated specific interactions with the secretory pathway lectins calnexin and ERGIC-53 PMID: 16938437
    32. Results indicate that ERGIC-53 can bind cargo glycoproteins in an MCFD2-independent fashion and suggest that MCFD2 is a recruitment factor for blood coagulation factors V and VIII. PMID: 17010120
    33. a mannose-binding lectin codon 54 gene polymorphism has a role in protection against Chlamydia trachomatis infection and Fallopian tube damage PMID: 17496053
    34. ERGIC-53 gene transcription is regulated in response to endoplasmic reticulum stress PMID: 17535801
    35. ERGIC-53 bound high-mannose-type oligosaccharides with low affinity and broad specificity, not discriminating between monoglucosylated and deglucosylated high-mannosetype oligosaccharides. PMID: 18025080
    36. Silencing Surf4 together with ERGIC-53 or silencing the p24 family member p25 induced an identical phenotype characterized by a reduced number of ERGIC clusters and fragmentation of the Golgi apparatus without effect on anterograde transport. PMID: 18287528
    37. MCFD2 may play a primary role in the export of FV and FVIII from the ER, with the impact of LMAN1 mediated indirectly through its interaction with MCFD2 PMID: 18391077
    38. Study shows that SUMF1 interacts with protein disulfide isomerase (PDI) and ERp44, two thioredoxin family members residing in the early secretory pathway, and with ERGIC-53, a lectin that shuttles between the ER and the Golgi. PMID: 18508857
    39. Data suggest that transient dimerization is an obligatory step in FGFR3 biosynthesis and that TDII/ERGIC-53 complex formation may function as a checkpoint for FGFR3 sorting downstream the endoplasmic reticulum. PMID: 18577465
    40. LMAN1 mutational inactivation is a frequent and early event potentially contributing to colorectal tumorigenesis. PMID: 19118014
    41. MBL deposition and gene expression in advanced human atherosclerotic lesions revealed the presence of MBL protein in ruptured but not stable atherosclerotic lesions PMID: 19380618
    42. Observational study of gene-gene interaction. (HuGE Navigator) PMID: 11333866

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  • 相关疾病：
    Factor V and factor VIII combined deficiency 1 (F5F8D1)
  • 亚细胞定位：
    Endoplasmic reticulum-Golgi intermediate compartment membrane; Single-pass type I membrane protein. Golgi apparatus membrane; Single-pass membrane protein. Endoplasmic reticulum membrane; Single-pass type I membrane protein.
  • 组织特异性：
    Ubiquitous.
  • 数据库链接：

    HGNC: 6631

    OMIM: 227300

    KEGG: hsa:3998

    STRING: 9606.ENSP00000251047

    UniGene: Hs.465295