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Rat Anti-MCFD2 Recombinant Antibody (CBXS-1757) (CBMAB-S4579-CQ)

This product is a rat antibody that recognizes MCFD2. The antibody CBXS-1757 can be used for immunoassay techniques such as: IHC.
See all MCFD2 antibodies

Summary

Host Animal
Rat
Specificity
Mouse
Clone
CBXS-1757
Antibody Isotype
IgG2a
Application
IHC

Basic Information

Immunogen
NS0-derived recombinant mouse SDNSF/MCFD2, His27-Gln145 (predicted), Accession # Q8K5B2
Specificity
Mouse
Antibody Isotype
IgG2a
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Lyophilized
Concentration
LYOPH
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
multiple coagulation factor deficiency 2
Introduction
This gene encodes a soluble luminal protein with two calmodulin-like EF-hand motifs at its C-terminus. This protein forms a complex with LMAN1 (lectin mannose binding protein 1; also known as ERGIC-53) that facilitates the transport of coagulation factors V (FV) and VIII (FVIII) from the endoplasmic reticulum to the Golgi apparatus via an endoplasmic reticulum Golgi intermediate compartment (ERGIC). Mutations in this gene cause combined deficiency of FV and FVIII (F5F8D); a rare autosomal recessive bleeding disorder characterized by mild to moderate bleeding and coordinate reduction in plasma FV and FVIII levels. This protein has also been shown to maintain stem cell potential in adult central nervous system and is a marker for testicular germ cell tumors. The 3' UTR of this gene contains a transposon-like human repeat element named 'THE 1'. A processed RNA pseudogene of this gene is on chromosome 6p22.1. Alternative splicing results in multiple transcript variants encoding distinct isoforms.
Entrez Gene ID
193813
UniProt ID
Q8K5B2
Alternative Names
1810021C21Rik; F5f8d; Lman1ip; Sdnsf
Function
The MCFD2-LMAN1 complex forms a specific cargo receptor for the ER-to-Golgi transport of selected proteins. Plays a role in the secretion of coagulation factors.
Biological Process
Protein transport Source: UniProtKB-KW
Vesicle-mediated transport Source: UniProtKB-KW
Cellular Location
Endoplasmic reticulum
Golgi apparatus
Other locations
Endoplasmic reticulum-Golgi intermediate compartment
Involvement in disease
Factor V and factor VIII combined deficiency 2 (F5F8D2):
A 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.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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