LMAN1 Antibody
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中文名稱：LMAN1兔多克隆抗體
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貨號：CSB-PA012991GA01HU
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規格：￥3,900
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其他：
產品詳情
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Uniprot No.：
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基因名：
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別名：LMAN1; ERGIC53; F5F8D; Protein ERGIC-53; ER-Golgi intermediate compartment 53 kDa protein; Gp58; Intracellular mannose-specific lectin MR60; Lectin mannose-binding 1
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宿主：Rabbit
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反應種屬：Human,Mouse,Rat
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免疫原：Human LMAN1
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免疫原種屬：Homo sapiens (Human)
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抗體亞型：IgG
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純化方式：Antigen Affinity Purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
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產品提供形式：Liquid
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應用范圍：ELISA,WB,IHC
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Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：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.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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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.
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基因功能參考文獻：
- 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
- 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
- Mannan-binding lectin reduces CpG DNA-induced inflammatory cytokine production in monocytes. PMID: 25664598
- MMP-9 secretion was reduced in the LMAN1 knockout cell line compared to control cells confirming the functional role of LMAN1. PMID: 26150355
- Authors 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
- 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
- Data indicate that together with its soluble coreceptor MCFD2, LMAN1 transports coagulation factors V (FV) and VIII (FVIII). PMID: 23709226
- 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
- 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
- Two new mutations at ERGIC-53 gene in a Turkish family. PMID: 20460353
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- surfactant proteins A and D and mannose-binding lectin play roles in inflammation caused by DNA in lungs and other tissues PMID: 15145932
- ERGIC-53 and MCFD2 have important functions during cellular response to stress conditions PMID: 15292203
- The ERGIC-53 is stationary and not simply a collection of mobile carriers that mediate protein traffic from endoplasmic reticulum to Golgi. PMID: 15632110
- 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
- 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
- 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
- Mutations in (LMAN1) and (MCFD2), have been found to be responsible for the dual deficiency of FV and FVIII. PMID: 16044454
- 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
- role in Aspergillus mediated allergies and infections PMID: 16114131
- ERGIC-53 is present exclusively as a hexameric complex in cells PMID: 16257008
- Mannan-binding lectin activates C3 and the alternative complement pathway without involvement of C2 PMID: 16670774
- 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
- Here, we have observed that NCT N-linked oligosaccharides mediated specific interactions with the secretory pathway lectins calnexin and ERGIC-53 PMID: 16938437
- 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
- a mannose-binding lectin codon 54 gene polymorphism has a role in protection against Chlamydia trachomatis infection and Fallopian tube damage PMID: 17496053
- ERGIC-53 gene transcription is regulated in response to endoplasmic reticulum stress PMID: 17535801
- ERGIC-53 bound high-mannose-type oligosaccharides with low affinity and broad specificity, not discriminating between monoglucosylated and deglucosylated high-mannosetype oligosaccharides. PMID: 18025080
- 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
- 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
- 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
- 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
- LMAN1 mutational inactivation is a frequent and early event potentially contributing to colorectal tumorigenesis. PMID: 19118014
- 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
- Observational study of gene-gene interaction. (HuGE Navigator) PMID: 11333866
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相關疾病：Factor V and factor VIII combined deficiency 1 (F5F8D1)
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亞細胞定位：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.
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組織特異性：Ubiquitous.
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數據庫鏈接：
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