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MCFD2 recombinant protein :: Multiple Coagulation Factor Deficiency 2 Recombinant Protein

Scan QR to view Datasheet Catalog #    MBS143883 MCFD2 recombinant protein
Unit / Price
0.005 mg  /  $140 +1 FREE 8GB USB
0.025 mg  /  $205 +1 FREE 8GB USB
1 mg  /  $2,235 +2 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

Multiple Coagulation Factor Deficiency 2 (MCFD2), Recombinant Protein

 Also Known As   

Recombinant Human Multiple Coagulation Factor Deficiency 2

 Product Synonym Names    MCFD2 Human; Multiple Coagulation Factor Deficiency 2 Human Recombinant; SDNSF; LMAN1IP; Multiple coagulation factor deficiency protein 2; Neural stem cell-derived neuronal survival protein; MCFD2; F5F8D; DKFZp686G21263
 Product Gene Name   

MCFD2 recombinant protein

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Sequence    MASMTGGQQM GRGSHMEEPA ASFSQPGSMG LDKNTVHDQE HIMEHLEGVI NKPEAEMSPQ ELQLHYFKMH DYDGNNLLDG LELSTAITHV HKEEGSEQAP LMSEDELINI IDGVLRDDDK NNDGYIDYAE FAKSLQ
 OMIM    607788
 3D Structure    ModBase 3D Structure for Q8NI22
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 Host    E Coli
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 Purity/Purification    Greater than 90.0% as determined by SDS-PAGE.
 Form/Format    The MCFD2 protein solution contains 20mM Tris-HCl, pH-7.5, 100mM NaCl and 10% glycerol.
Sterile filtered colorless solution.
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 Preparation and Storage    Store at 4 degree C if entire vial will be used within 2-4 weeks. Store, frozen at -20 degree C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Avoid multiple freeze-thaw cycles.
 Other Notes    Small volumes of MCFD2 recombinant protein 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.
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Related Product Information for MCFD2 recombinant protein

   Description: MCFD2 Human Recombinant produced in E Coli is a single, non-glycosylated polypeptide chain containing 136 amino acids (27-146 a.a.) and having a molecular wieght of 20.9kDa. The MCFD2 is is fused to 16 a.a. T7-Tag at N-terminus and purified by proprietary chromatographic techniques.

Introduction: The MCFD2-LMAN1 complex forms an explicit cargo receptor for the ER-to-Golgi transport of selected proteins. MCFD2 is involved in the secretion of coagulation factors. MCFD2 is expressed by neural stem/progenitor cells of the hippocampus, and localized to region where neurogenesis persists throughout life. MCFD2 prevents NSC cell death and maintains stem cell characteristics. MCFD2 forms a complex with LAMN1 that facilitates the transport of coagulation factors V and VIII from the endoplasmic reticulum to the Golgi apparatus through an endoplasmic reticulum Golgi intermediate compartment. Mutations in the MCFD2 cause Factor V and Factor VIII combined deficiency.
 Product Categories/Family for MCFD2 recombinant protein    RECOMBINANT & NATURAL PROTEINS; Recombinant Proteins; Coagulation Factors
NCBI/Uniprot data below describe general gene information for MCFD2. It may not necessarily be applicable to this product.
 NCBI GI #    284005451
 NCBI GeneID    90411
 NCBI Accession #    NP_001164977.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_001171506.2 [Other Products]
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 UniProt Primary Accession #    Q8NI22 [Other Products]
 UniProt Secondary Accession #    Q53SS3; Q68D61; Q8N3M5; A8K7W2; D6W5A9; E9PD95 [Other Products]
 UniProt Related Accession #    Q8NI22 [Other Products]
 Molecular Weight    14,431 Da
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 NCBI Official Full Name    multiple coagulation factor deficiency protein 2 isoform A
 NCBI Official Synonym Full Names    multiple coagulation factor deficiency 2
 NCBI Official Symbol    MCFD2 [Similar Products]
 NCBI Official Synonym Symbols   
F5F8D; SDNSF; F5F8D2; LMAN1IP
[Similar Products]
 NCBI Protein Information    multiple coagulation factor deficiency protein 2; neural stem cell-derived neuronal survival protein
 UniProt Protein Name    Multiple coagulation factor deficiency protein 2
 UniProt Synonym Protein Names   
Neural stem cell-derived neuronal survival protein
 Protein Family    Multiple coagulation factor deficiency protein
 UniProt Gene Name    MCFD2 [Similar Products]
 UniProt Synonym Gene Names    SDNSF [Similar Products]
 UniProt Entry Name    MCFD2_HUMAN
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 NCBI Summary for MCFD2    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]
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 UniProt Comments for MCFD2    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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 Research Articles on MCFD2    1. Studies indicate that the LMAN1-CRD contains distinct, separable binding sites for both its partner protein MCFD2 and the cargo proteins FV/FVIII.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with MCFD2 recombinant proteinPathways associated with MCFD2 recombinant protein
 Reference Product  PubMed Publications
 LMAN1 recombinant protein  >43 publications with MCFD2 and LMAN1
 CTSZ recombinant protein  >4 publications with MCFD2 and CTSZ
 LMAN2 recombinant protein  >1 publications with MCFD2 and LMAN2
 Products by Pathway  Pathway Diagram
 Asparagine N-linked Glycosylation Pathway antibodies  Asparagine N-linked Glycosylation Pathway Diagram
 Defective ALG1 Causes ALG1-CDG (CDG-1k) Pathway antibodies  Defective ALG1 Causes ALG1-CDG (CDG-1k) Pathway Diagram
 Defective ALG11 Causes ALG11-CDG (CDG-1p) Pathway antibodies  Defective ALG11 Causes ALG11-CDG (CDG-1p) Pathway Diagram
 Defective ALG12 Causes ALG12-CDG (CDG-1g) Pathway antibodies  Defective ALG12 Causes ALG12-CDG (CDG-1g) Pathway Diagram
 Defective ALG14 Causes Congenital Myasthenic Syndrome (ALG14-CMS) Pathway antibodies  Defective ALG14 Causes Congenital Myasthenic Syndrome (ALG14-CMS) Pathway Diagram
 Defective ALG2 Causes ALG2-CDG (CDG-1i) Pathway antibodies  Defective ALG2 Causes ALG2-CDG (CDG-1i) Pathway Diagram
 Defective ALG3 Causes ALG3-CDG (CDG-1d) Pathway antibodies  Defective ALG3 Causes ALG3-CDG (CDG-1d) Pathway Diagram
 Defective ALG6 Causes ALG6-CDG (CDG-1c) Pathway antibodies  Defective ALG6 Causes ALG6-CDG (CDG-1c) Pathway Diagram
 Defective ALG8 Causes ALG8-CDG (CDG-1h) Pathway antibodies  Defective ALG8 Causes ALG8-CDG (CDG-1h) Pathway Diagram
 Defective ALG9 Causes ALG9-CDG (CDG-1l) Pathway antibodies  Defective ALG9 Causes ALG9-CDG (CDG-1l) Pathway Diagram
Diseases associated with MCFD2 recombinant proteinOrgans/Tissues associated with MCFD2 recombinant protein
 Disease Name  Pubmed Publications
 FACTOR V AND FACTOR VIII, COMBINED DEFICIENCY OF, 2 Antibodies  >17 publications with MCFD2 and FACTOR V AND FACTOR VIII, COMBINED DEFICIENCY OF, 2
 FACTOR V AND FACTOR VIII, COMBINED DEFICIENCY OF, 1 Antibodies  >13 publications with MCFD2 and FACTOR V AND FACTOR VIII, COMBINED DEFICIENCY OF, 1
 Anemia Antibodies  >2 publications with MCFD2 and Anemia
 Ovarian Diseases Antibodies  >1 publications with MCFD2 and Ovarian Diseases
 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >27 publications with MCFD2 and Blood
 Liver Antibodies  >2 publications with MCFD2 and Liver
 Bone Antibodies  >1 publications with MCFD2 and Bone
 Ovary Antibodies  >1 publications with MCFD2 and Ovary
 Testis Antibodies  >1 publications with MCFD2 and Testis
 Brain Antibodies  >1 publications with MCFD2 and Brain
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