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

Rabbit

1.0mg/ml (lot specific)

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.

Western Blot (WB)

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; ER to Golgi transport vesicle; ER-Golgi intermediate compartment; ER-Golgi intermediate compartment membrane; membrane

Molecular Function: protein binding; unfolded protein binding

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

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

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