LMAN1 antibody | MyBioSource MBS835014 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

LMAN1 antibody

catalog :

MBS835014

quantity :

0.1 mL

price :

515 USD

clonality :

monoclonal

host :

mouse

conjugate :

nonconjugated

clone name :

2B10

reactivity :

human

application :

western blot, immunocytochemistry

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS835014

products type :

Antibody

products full name :

LMAN1 antibody

products short name :

LMAN1

products name syn :

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

other names :

protein ERGIC-53; Protein ERGIC-53; protein ERGIC-53; lectin, mannose-binding, 1; ER-Golgi intermediate compartment 53 kDa protein; Gp58; Intracellular mannose-specific lectin MR60; Lectin mannose-binding 1

products gene name :

LMAN1

other gene names :

LMAN1; LMAN1; MR60; gp58; F5F8D; FMFD1; MCFD1; ERGIC53; ERGIC-53; ERGIC53; F5F8D

uniprot entry name :

LMAN1_HUMAN

clonality :

Monoclonal

isotype :

IgG1

clone :

2B10

host :

Mouse

reactivity :

Human, Monkey

sequence length :

510

purity :

LMAN1 antibody was purified by affinity chromatography.

form :

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

concentration :

500 ug-1 mg/ml

storage stability :

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.

tested application :

Immunofluorescence (IF), Western Blot (WB)

app notes :

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

other info1 :

Biological Significance: LMAN1 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 its gene are associated with a coagulation defect. Using positional cloning, its 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.

other info2 :

Immunogen: LMAN1 antibody was raised in mouse using a full length recombinant protein of human LMAN1 (NP_005561) produced in HEK293T cells, as the immunogen.

products categories :

Cell Biology

products description :

Mouse monoclonal LMAN1 antibody

ncbi gi num :

5031873

ncbi acc num :

NP_005561

ncbi gb acc num :

NM_005570.3

uniprot acc num :

P49257

ncbi mol weight :

57,549 Da

ncbi pathways :

Asparagine N-linked Glycosylation Pathway (161013); Metabolism Of Proteins Pathway (106230); Post-translational Protein Modification Pathway (161012); Protein Processing In Endoplasmic Reticulum Pathway (167325); Protein Processing In Endoplasmic Reticulum Pathway (167190); Signaling Events Mediated By TCPTP Pathway (169350); Transport To The Golgi And Subsequent Modification Pathway (187220)

ncbi summary :

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]

uniprot summary :

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

size1 :

0.1 mL

price1 :

515 USD

more info or order :

MyBioSource product webpage