DATASHEET
Host:
Rabbit
Target Protein:
MCFD2
Immunogen Range:
1-100/145
Clonality:
Polyclonal
Isotype:
IgG
Entrez Gene:
193813
Swiss Prot:
Q8K5B2
Source:
KLH conjugated synthetic peptide derived from mouse MCFD2
Purification:
Purified by Protein A.
Storage Buffer:
0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Storage:
Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
Background:
This gene encodes a soluble luminal protein with two calmodulin-like EF-hand motifs at its C-terminus. This protein forms a complex with LAMN1 (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. [provided by RefSeq, Jun 2010]
VALIDATION IMAGES
Paraformaldehyde-fixed, paraffin embedded Rat brain; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with MCFD2 Polyclonal Antibody, Unconjugated (bs-24080R) at 1:400 overnight at 4°C, DAB staining.
Lane 1: Mouse Heart lysates; Lane 2:Rat Heart lysates probed with MCFD2 Polyclonal Antibody, Unconjugated (bs-24080R) at 1:500 dilution and 4˚C overnight incubation. Followed by conjugated secondary antibody incubation at 1:20000 for 60 min at 37˚C.