Monoclonal LMAN1; Anti-LMAN1; Lectin Mannose-Binding 1; ERGIC-53; ERGIC53; F5F8D; FMFD1; MCFD1; MR60; gp58; LMAN1; LMAN 1; LMAN-1

For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

IgG1

2B10

Mouse

LMAN1 antibody was purified by affinity chromatography.

Supplied in PBS buffer, pH 7.3, containing 1% BSA, 50% glycerol and 0.02% sodium azide.

500 ug-1 mg/ml (lot specific)

Store at 4 degree C for short term storage. Aliquot and store at -20 degree C for long term storage. Avoid repeated freeze/thaw cycles.

Small volumes of anti-LMAN1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Mouse monoclonal LMAN1 antibody

Cell Biology

Immunofluorescence (IF), Western Blot (WB)

IF: 1:100
WB: 1:200-500

Western Blot analysis of HEK293T cell lysates (5 ug) transfected with either recombinant LMAN1 protein (Right) or empty vector (Left) detected with LMAN1 antibody

57,549 Da

lectin, mannose-binding, 1

protein ERGIC-53

Protein ERGIC-53

ERGIC53; F5F8D??[Similar Products]

LMAN1_HUMAN

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]

LMAN1: 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. Defects in LMAN1 are THE cause of factor V and factor VIII combined deficiency type 1 (F5F8D1); 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.

Protein type: Membrane protein, integral

Chromosomal Location of Human Ortholog: 18q21.3-q22

Cellular Component: Golgi membrane; endoplasmic reticulum membrane; ER-Golgi intermediate compartment membrane; sarcomere; membrane; ER-Golgi intermediate compartment; integral to membrane

Molecular Function: mannose binding; protein binding; metal ion binding; unfolded protein binding

Biological Process: endoplasmic reticulum organization and biogenesis; ER to Golgi vesicle-mediated transport; cellular protein metabolic process; protein folding; protein exit from endoplasmic reticulum; protein amino acid N-linked glycosylation via asparagine; post-translational protein modification; blood coagulation; Golgi organization and biogenesis

Disease: Factor V And Factor Viii, Combined Deficiency Of, 1

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