Overview

Description

  • Nature
    Native
  • Source
    Native
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVI SKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTI QPTQPTTQLPTDSPTQPTTG SFCPGPVTLCSDLESHSTEAVLGDALVD FSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILS YPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSP RVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAK WKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQ LSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTL PRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLE LTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDP RA
    • Molecular weight
      100 kDa
    • Amino acids
      1 to 500
    • Additional sequence information
      ab183265 is prepared from Human plasma.

Specifications

Our Abpromise guarantee covers the use of ab183265 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    SDS-PAGE

  • Purity
    >95% by SDS-PAGE .

  • Form
    Liquid
  • Additional notes

    Extinction coefficient 0.4.

    ab183265 is shown to be non reactive for HbsAG, anti-HCV, anti-HBc, and negative for anti-HIV 1 & 2 by FDA approved tests.

  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on Dry Ice. Store at -80°C.

    pH: 7
    Constituents: 0.27% Potassium phosphate, 1.46% Sodium chloride

General Info

  • Alternative names
    • C1 esterase inhibitor
    • C1 Inh
    • C1 inhibiting factor
    • C1 inhibitor
    • C1-inhibiting factor
    • C1IN
    • C1Inh
    • C1NH
    • complement component 1 inhibitor
    • esterase inhibitor
    • HAE1
    • HAE2
    • IC1_HUMAN
    • Plasma protease C1 inhibitor
    • Serine (or cysteine) proteinase inhibitor clade G member 1
    • serine/cysteine proteinase inhibitor clade G member 1
    • Serpin G1
    • serpin peptidase inhibitor, clade G (C1 inhibitor), member 1
    • SERPING1
    see all
  • 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.
  • 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.
  • Sequence similarities
    Belongs to the serpin family.
  • Post-translational
    modifications
    Highly glycosylated (49%) with N- and O-glycosylation.
    Can be proteolytically cleaved by E.coli stcE.
  • Cellular localization
    Secreted.
  • Information by UniProt

Images

  • 5% Tris HCl gel, 200V, 45 min.

    Lane 1: 10 ug of ab183265, unheated.

    Lane 2: 10 ug of ab183265, heated.

References

ab183265 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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