F5F8D; FMFD1; MCFD1; MR60; Gp58; Protein ERGIC-53; Coagulation Factor V-Factor VIII Combined Deficiency; Endoplasmic Reticulum-Golgi Intermediate Compartment Protein 53

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

Polyclonal

IgG

Rabbit

The antibody is a rabbit polyclonal antibody raised against LMAN1. It has been selected for its ability to recognize LMAN1 in immunohistochemical staining and western blotting.

Affinity Chromatography

Supplied as solution form in PBS, pH7.4 containing 0.02% NaN3, 50% glycerol.

200ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C to -80 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

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.

Knock-Out Validation Antibody

Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA)

Western Blot: 1:50-400
Immunohistochemistry in formalin fixed frozen section: 1:50-500
Enzyme-linked Immunosorbent Assay: 1:100-200

Western Blot: Sample: Human Hela cell lysate; Primary Ab: 1ug/ml Rabbit Anti-Human LMAN1 Antibody Second Ab: 0.2ug/mL?HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody

Western Blot: Sample: Recombinant protein.

DAB staining on fromalin fixed paraffin- embedded Kidney tissue)

Knockout Validation: Lane 1: Wild-type Hela cell lysate; Lane 2: LMAN1 knockout Hela cell lysate; Predicted MW: 58kd Observed MW: 58kd Primary Ab: 1ug/ml Rabbit Anti-Human LMAN1 Antibody Second Ab: 0.2ug/mL?HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody (#MBS2086047)

57,549 Da

lectin, mannose binding 1

protein ERGIC-53

Protein ERGIC-53

ERGIC53; F5F8D??[Similar Products]

The protein encoded by this gene is a membrane mannose-specific lectin that cycles between the endoplasmic reticulum, endoplasmic reticulum-Golgi intermediate compartment, and cis-Golgi, functioning as a cargo receptor for glycoprotein transport. The protein has an N-terminal signal sequence, a calcium-dependent and pH-sensitive carbohydrate recognition domain, a stalk region that functions in oligomerization, a transmembrane domain, and a short cytoplasmic domain required for organelle targeting. Allelic variants of this gene are associated with the autosomal recessive disorder combined factor V-factor VIII deficiency. [provided by RefSeq, Jul 2015]

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: 18q21.32

Cellular Component: endoplasmic reticulum membrane; endoplasmic reticulum-Golgi intermediate compartment; endoplasmic reticulum-Golgi intermediate compartment membrane; Golgi membrane; integral component of membrane; membrane; sarcomere

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

Biological Process: blood coagulation; COPII vesicle coating; endoplasmic reticulum organization; ER to Golgi vesicle-mediated transport; Golgi organization and biogenesis; protein exit from endoplasmic reticulum; protein folding; protein N-linked glycosylation via asparagine

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

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