Anti -MCFD2, NT (Neural Stem Cell-derived Neuronal Survival Protein, Multiple Coagulation Factor Deficiency Protein 2, SDNSF)

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 2; NC_000002.11 (47129009..47168994, complement). Location: 2p21

Polyclonal

IgG

Rabbit

Recognizes human MCFD2.

Affinity Purified
Purified by Protein A affinity chromatography.

Supplied as a liquid in PBS, 0.09% sodium azide.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Small volumes of anti-MCFD2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

The MCFD2-LMAN1 complex forms a specific cargo receptor for the ER-to-Golgi transport of selected proteins. MCFD2 interacts in a calcium-dependent mannder with LMAN1. Defects in MCFD2 are a cause of factor V and factor VIII combined deficiency (F5F8D); also known as multiple coagulation factor deficiency 2 (MCFD2). F5F8D is an autosomal recessive bleeding disorder characterized by coordinate reduction of both clotting proteins.

Antibodies; Abs to Coagulation Factors

ELISA (EL/EIA), Immunohistochemistry (IHC)

Suitable for use in ELISA and Immunohistochemistry.
Dilution: ELISA: 1:1,000
Immunohistochemistry: 1:10-1:50

10,718 Da[Similar Products]

multiple coagulation factor deficiency 2

multiple coagulation factor deficiency protein 2; OTTHUMP00000203114; OTTHUMP00000203115; OTTHUMP00000203116; OTTHUMP00000203117; OTTHUMP00000203118; OTTHUMP00000203119; OTTHUMP00000203120; OTTHUMP00000203121; neural stem cell derived neuronal survival protein; neural stem cell-derived neuronal survival protein

MCFD2 protein

MCFD2??[Similar Products]

Q68D61_HUMAN

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 ***** 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]

MCFD2: 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. Defects in MCFD2 are a cause of factor V and factor VIII combined deficiency type 2 (F5F8D2); also known as multiple coagulation factor deficiency 2 (MCFD2). F5F8D2 is 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. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Endoplasmic reticulum

Chromosomal Location of Human Ortholog: 2p21

Cellular Component: Golgi apparatus; endoplasmic reticulum; ER-Golgi intermediate compartment

Molecular Function: calcium ion binding

Biological Process: vesicle-mediated transport; protein transport; cellular protein metabolic process; protein amino acid N-linked glycosylation via asparagine; post-translational protein modification

Disease: Factor V And Factor Viii, Combined Deficiency Of, 2

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