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Rabbit Anti-LMAN1 antibody
Rabbit Anti-LMAN1 antibody
Endoplasmic reticulum golgi intermediate compartment protein 53; ER-Golgi intermediate compartment 53 kDa protein; ERGIC-53; ERGIC53; ERGIC53 like protein; F5F8D; FMFD1; Gp58; Intracellular mannose specific lectin; Intracellular mannose-specific lectin MR
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  • NO.:SL18304R
    Clonality:Polyclonal
    Immunogen Species:Rabbit
    React Species:Human,(predicted: Mouse,Rat,Dog,Cow,Horse,Rabbit,Sheep,)
    Applications:WB ELISA IHC-P IHC-F ICC IF
    concentration:1mg/ml
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Product Name LMAN1
Chinese Name 凝集素甘露糖Binding protein1抗体
Alias Endoplasmic reticulum golgi intermediate compartment protein 53; ER-Golgi intermediate compartment 53 kDa protein; ERGIC-53; ERGIC53; ERGIC53 like protein; F5F8D; FMFD1; Gp58; Intracellular mannose specific lectin; Intracellular mannose-specific lectin MR60; Lectin mannose binding 1; Lectin mannose-binding 1; Lman1; LMAN1 like protein; LMAN1_HUMAN; MCFD1; MR60; Protein ERGIC-53.  
Research Area Cell biology  immunology  Signal transduction  
Immunogen Species Rabbit
Clonality Polyclonal
React Species Human,  (predicted: Mouse, Rat, Dog, Cow, Horse, Rabbit, Sheep, )
Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight 54kDa
Cellular localization cytoplasmic 
Form Liquid
Concentration 1mg/ml
immunogen KLH conjugated synthetic peptide derived from human LMAN1: 31-130/510 
Lsotype IgG
Purification affinity purified by Protein A
Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
Product Detail The protein encoded by this gene is a type I integral membrane protein localized in the intermediate region between the endoplasmic reticulum and the Golgi, presumably recycling between the two compartments. The protein is a mannose-specific lectin and is a member of a novel family of plant lectin homologs in the secretory pathway of animal cells. Mutations in the gene are associated with a coagulation defect. Using positional cloning, the gene was identified as the disease gene leading to combined factor V-factor VIII deficiency, a rare, autosomal recessive disorder in which both coagulation factors V and VIII are diminished. [provided by RefSeq, Jul 2008]

Function:
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.

Subcellular Location:
Endoplasmic reticulum-Golgi intermediate compartment membrane. Golgi apparatus membrane. Endoplasmic reticulum membrane.

Tissue Specificity:
Ubiquitous.

Post-translational modifications:
The N-terminal may be partly blocked.

DISEASE:
Defects in LMAN1 are THE cause of factor V and factor VIII combined deficiency type 1 (F5F8D1) [MIM:227300]; also known as multiple coagulation factor deficiency I (MCFD1). F5F8D1 is an autosomal recessive blood coagulation disorder characterized by bleeding symptoms similar to those in hemophilia or parahemophilia, that are caused by single deficiency of FV or FVIII, respectively. The most common symptoms are epistaxis, menorrhagia, and excessive bleeding during or after trauma. Plasma levels of coagulation factors V and VIII are in the range of 5 to 30% of normal.

Similarity:
Contains 1 L-type lectin-like domain.

SWISS:
P49257

Gene ID:
3998

Database links:

Entrez Gene: 3998 Human

Entrez Gene: 70361 Mouse

Entrez Gene: 116666 Rat

Omim: 601567 Human

SwissProt: P49257 Human

SwissProt: Q9D0F3 Mouse

SwissProt: Q62902 Rat

Unigene: 465295 Human

Unigene: 290857 Mouse

Unigene: 449042 Mouse

Unigene: 25734 Rat



Product Picture
Tissue/cell: human kidney tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-LMAN1 Polyclonal Antibody, Unconjugated(SL18304R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
Tissue/cell: human prostate tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-LMAN1 Polyclonal Antibody, Unconjugated(SL18304R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining

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