Recombinant Human ANTXR2 protein (ab153587)

Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      GRGFMLGSRNGSVLCTYTVNETYTTSVKPVSVQLNSMLCPAPILNKAGET LDVSVSFNGGKSVISGSLIV
    • Amino acids
      241 to 310
    • Tags
      proprietary tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab153587 in the following tested applications.

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

  • Applications

    Western blot

    ELISA

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 mg/ml.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names
    • 2310046B19Rik
    • Anthrax toxin receptor 2
    • ANTR2_HUMAN
    • Antxr2
    • AW561899
    • Capillary morphogenesis gene 2 protein
    • Capillary morphogenesis protein 2
    • CMG 2
    • CMG-2
    • CMG2
    • FLJ31074
    • HFS
    • ISH
    • JHF
    • MGC111533
    • MGC45856
    see all
  • Function
    Necessary for cellular interactions with laminin and the extracellular matrix.
  • Tissue specificity
    Expressed in prostate, thymus, ovary, testis, pancreas, colon, heart, kidney, lung, liver, peripheral blood leukocytes, placenta, skeletal muscle, small intestine and spleen.
  • Involvement in disease
    Defects in ANTXR2 are the cause of infantile systemic hyalinosis (ISH) [MIM:236490]. This autosomal recessive syndrome is similar to JHF, but has an earlier onset and a more severe course. Symptoms appear at birth or within the first months of life, with painful, swollen joint contractures, osteopenia, osteoporosis and livid red hyperpigmentation over bony prominences. Patients develop multiple subcutaneous skin tumors and gingival hypertrophy. Hyaline deposits in multiple organs, recurrent infections and intractable diarrhea often lead to death within the first 2 years of life. Surviving children may suffer from severely reduced mobility due to joint contractures.
    Defects in ANTXR2 are the cause of juvenile hyaline fibromatosis (JHF) [MIM:228600]. JHF is an autosomal recessive syndrome that is similar to ISH but takes a milder course. It is characterized by hyaline deposition in the dermis, multiple subcutaneous skin tumors and gingival hypertrophy, followed by progressive joint contractions, osteopenia and osteoporosis that may lead to a severe limitation of mobility.
  • Sequence similarities
    Belongs to the ATR family.
    Contains 1 VWFA domain.
  • Domain
    Binding to PA seems to be effected through the VWA domain.
  • Cellular localization
    Secreted; Cell membrane. Expressed at the cell surface and Endoplasmic reticulum membrane. Expressed predominantly within the endoplasmic reticulum and not at the plasma membrane.
  • Information by UniProt

Images

  • ab153587 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab153587 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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