Mouse Anti-MCFD2 (AA 1-146) Recombinant Antibody (CBFYM-1869) (CBMAB-M2040-FY)

Name: Mouse Anti-MCFD2 (AA 1-146) Recombinant Antibody (CBFYM-1869)
SKU: CBMAB-M2040-FY
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

CBFYM-1869

Application

ELISA, IF, WB

Immunogen

Full length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.Immunogen sequence: MTMRSLLRTP FLCGLLWAFC APGARAEEPA ASFSQPGSMG LDKNTVHDQE HIMEHLEGVI NKPEAEMSPQ ELQLHYFKMH DYDGNNLLDG LELSTAITHV HKEEGSEQAP LMSEDELINI IDGVLRDDDK NNDGYIDYAE F

Specificity

Human

Antibody Isotype

IgG1, k

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

Liquid

Storage

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

Epitope

AA 1-146

More Infomation

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 that facilitates the transport of coagulation factors V and VIII from the endoplasmic reticulum to the Golgi apparatus via an endoplasmic reticulum Golgi intermediate compartment. Mutations in this gene cause combined deficiency of FV and FVIII; 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

90411

UniProt ID

Q8NI22

Alternative Names

Multiple Coagulation Factor Deficiency 2; Neural Stem Cell-Derived Neuronal Survival Protein; SDNSF; Multiple Coagulation Factor Deficiency Protein 2; LMAN1IP; F5F8D2; F5F8D

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.

Watanabe, S., Kise, Y., Yonezawa, K., Inoue, M., Shimizu, N., Nureki, O., & Inaba, K. (2023). Structure of full-length ERGIC-53 in complex with MCFD2 for cargo transport. bioRxiv, 2023-08.

Zhang, Y., Liu, Z., & Zhang, B. (2023). Separate roles of LMAN1 and MCFD2 in ER-to-Golgi trafficking of FV and FVIII. Blood Advances, 7(7), 1286-1296.

Kirimoto, Y., Yamano-Adachi, N., Koga, Y., & Omasa, T. (2023). Effect of co-overexpression of the cargo receptor ERGIC-53/MCFD2 on antibody production and intracellular IgG secretion in recombinant Chinese hamster ovary cells. Journal of bioscience and bioengineering, 136(5), 400-406.

Zhang, Y., Zhu, M., Zheng, C., Wei, W., Emmer, B. T., & Zhang, B. (2022). LMAN1–MCFD2 complex is a cargo receptor for the ER-Golgi transport of α1-antitrypsin. Biochemical Journal, 479(7), 839-855.

Zhang, Y., Liu, Z., & Zhang, B. (2022). Separate roles of LMAN1 and MCFD2 in ER-to-Golgi trafficking of factor V and factor VIII. bioRxiv, 2022-09.

Satoh, T., Nishio, M., Suzuki, K., Yagi-Utsumi, M., Kamiya, Y., Mizushima, T., & Kato, K. (2020). Crystallographic snapshots of the EF-hand protein MCFD2 complexed with the intracellular lectin ERGIC-53 involved in glycoprotein transport. Acta Crystallographica Section F: Structural Biology Communications, 76(5), 216-221.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

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Isotype control

For research use only. Not intended for any clinical use.

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