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| Reactivity: | Human, Mouse, Rat |
| Applications: | WB, IHC, ELISA |
| Host Species: | Rabbit |
| Clonality: | Polyclonal antibody |
| Gene Name: | multiple coagulation factor deficiency 2, ER cargo receptor complex subunit |
| Gene Symbol: | MCFD2 |
| Synonyms: | F5F8D; SDNSF; F5F8D2; LMAN1IP; MCFD2 |
| Gene ID: | 90411 |
| UniProt ID: | Q8NI22 |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 27-146 of human MCFD2 (NP_644808.1). |
| Dilution: | WB 1:500-1:2000; IHC 1:100-1:200 |
| Purification Method: | Affinity purification |
| Concentration: | 2.32 mg/mL |
| Buffer: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Storage: | Store at -20°C. Avoid freeze / thaw cycles. |
| Documents: | Manual-MCFD2 antibody |
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 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.
Images
 | Western blot analysis of various lysates using MCFD2 Rabbit pAb (A10376) at 1:1000 dilution. Secondary antibody: HRP-conjugated Goat anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution. Lysates/proteins: 25μg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s. |
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