Human Protein ERGIC-53 (LMAN1) ELISA kit; ERGIC-53; ERGIC53; F5F8D; FMFD1; MCFD1; MR60; gp58; endoplasmic reticulum-golgi intermediate compartment protein 53; intracellular mannose specific lectin; lectin; mannose-binding; 1

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

Store all reagents at 2-8 degree C

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Small volumes of LMAN1 elisa kit 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.

MBS9328074 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the lectin, mannose-binding, 1 (LMAN1) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing LMAN1. The ELISA analytical biochemical technique of the MBS9328074 kit is based on LMAN1 antibody-LMAN1 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect LMAN1 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, LMAN1. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

57,549 Da

lectin, mannose-binding, 1

protein ERGIC-53; intracellular mannose specific lectin; intracellular mannose-specific lectin MR60; ER-Golgi intermediate compartment 53 kDa protein; endoplasmic reticulum-golgi intermediate compartment protein 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; sarcomere; ER-Golgi intermediate compartment membrane; membrane; integral to membrane; ER-Golgi intermediate compartment

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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