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SERPING1 native protein :: C1 Inhibitor (Esterase Inhibitor) Native Protein

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Catalog # MBS318174
Unit / Price
  1 mg  /  $295 +1 FREE 8GB USB
SERPING1 native protein
Product Name

C1 Inhibitor (Esterase Inhibitor) (SERPING1), Native Protein

Popular Item
Also Known As

C1 Esterase Inhibitor

Product Synonym Names
Human C1 Esterase Inhibitor
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Chromosome Location
Chromosome: 11; NC_000011.9 (57365027..57382326). Location: 11q12-q13.1
OMIM
106100
3D Structure
ModBase 3D Structure for P05155
Host
Human, Plasma
Purity/Purification
>95% pure (SDS-PAGE)
Form/Format
Purified, Liquid
Concentration
2.14 mg/ml (lot specific)
Buffer
20mM Potassium phosphate, pH 7.0 containing 250mM Potassium chloride
Preservative
None
Preparation and Storage
Store at -70°C.
Avoid multiple freeze/thaw cycles
Shipping: Ships with dry ice
ISO Certification
Manufactured in an ISO 9001:2008 Certified Laboratory.
Other Notes
Small volumes of SERPING1 native 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.
Related Product Information for
SERPING1 native protein
C1 Esterase Inhibitor
Human C1 Esterase Inhibitor
Product Categories/Family for SERPING1 native protein
Applications Tested/Suitable for SERPING1 native protein
Specific methodologies have not been tested using this product
NCBI/Uniprot data below describe general gene information for SERPING1. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
55,154 Da[Similar Products]
NCBI Official Full Name
C1 inhibitor
NCBI Official Synonym Full Names
serpin peptidase inhibitor, clade G (C1 inhibitor), member 1
NCBI Official Symbol
SERPING1  [Similar Products]
NCBI Official Synonym Symbols
C1IN; C1NH; HAE1; HAE2; C1INH
  [Similar Products]
NCBI Protein Information
plasma protease C1 inhibitor; serpin G1; OTTHUMP00000197003; OTTHUMP00000197004; OTTHUMP00000197007; OTTHUMP00000235286; OTTHUMP00000235287; C1-inhibiting factor; C1 esterase inhibitor; complement component 1 inhibitor; serine/cysteine proteinase inhibitor clade G member 1
UniProt Protein Name
Plasma protease C1 inhibitor
UniProt Synonym Protein Names
C1 esterase inhibitor; C1-inhibiting factor; Serpin G1
UniProt Gene Name
SERPING1  [Similar Products]
UniProt Synonym Gene Names
C1IN; C1NH  [Similar Products]
UniProt Entry Name
IC1_HUMAN
NCBI Summary for SERPING1
This gene encodes a highly glycosylated plasma protein involved in the regulation of the complement cascade. Its protein inhibits activated C1r and C1s of the first complement component and thus regulates complement activation. Deficiency of this protein is associated with hereditary angioneurotic oedema (HANE). Alternative splicing results in multiple transcript variants encoding the same isoform. [provided by RefSeq]
UniProt Comments for SERPING1
Function: Activation of the C1 complex is under control of the C1-inhibitor. It forms a proteolytically inactive stoichiometric complex with the C1r or C1s proteases. May play a potentially crucial role in regulating important physiological pathways including complement activation, blood coagulation, fibrinolysis and the generation of kinins. Very efficient inhibitor of FXIIa. Inhibits chymotrypsin and kallikrein. Ref.21

Subunit structure: Binds to E.coli stcE which allows localization of SERPING1 to cell membranes thus protecting the bacteria against complement-mediated lysis. Interacts with MASP1. Ref.22 Ref.23 Ref.25

Subcellular location: Secreted.

Post-translational modification: Highly glycosylated (49%) with N- and O-glycosylation. Ref.2 Ref.24 Ref.26 Ref.27 Ref.28 Ref.29 Ref.30 Ref.31Can be proteolytically cleaved by E.coli stcE. Ref.23 Ref.25

Polymorphism: Chymotrypsin uses Ala-465 as its reactive site in normal plasma protease C1 inhibitor, and His-466 as its reactive site in the variant His-466.

Involvement in disease: Defects in SERPING1 are the cause of hereditary angioedema (HAE) [

MIM:106100]; also called hereditary angioneurotic edema (HANE). HAE is an autosomal dominant disorder characterized by episodic local subcutaneous edema and submucosal edema involving the upper respiratory and gastrointestinal tracts. HAE due to C1 esterase inhibitor deficiency is comprised of two clinically indistinguishable forms. In HAE type 1, representing 85% of patients, serum levels of C1 esterase inhibitor are less than 35% of normal. In HAE type 2, the levels are normal or elevated, but the protein is non-functional. Ref.14 Ref.20 Ref.33 Ref.34 Ref.35 Ref.36 Ref.37 Ref.38 Ref.39 Ref.40 Ref.41 Ref.42 Ref.43 Ref.44 Ref.45

Sequence similarities: Belongs to the serpin family.

Sequence caution: The sequence AAA53096.1 differs from that shown. Reason: Erroneous gene model prediction.
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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