Overview

Description

  • Nature

    Recombinant
  • Source

    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Sequence

      MLGEEHTRSIGPRESAKEYRQRSLLEKELLFFAYDVFGIPFVDPDSWTPE EVIPKRLQEKQKSERETAVRISQEIGNLMKEIETLVEEKTKESLDVSRLT REGGPLLYEGISLTMNSKLLNGSQRVVMDGVISDHECQELQRLTNVAATS GDGYRGQTSPHTPNEKFYGVTVFKALKLGQEGKVPLQSAHLYYNVTEKVR RIMESYFRLDTPLYFSYSHLVCRTAIEEVQAERKDDSHPVHVDNCILNAE TLVCVKEPPAYTFRDYSAILYLNGDFDGGNFYFTELDAKTVTAEVQPQCG RAVGFSSGTENPHGVKAVTRGQRCAIALWFTLDPRHSERDRVQADDLVKM LFSPEEMDLSQEQPLDAQQGPPEPAQESLSGSESKPKDEL
    • Molecular weight

      69 kDa including tags
    • Amino acids

      347 to 736

Specifications

Our Abpromise guarantee covers the use of ab132600 in the following tested applications.

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

  • Applications

    ELISA

    SDS-PAGE

    Western blot

  • Form

    Liquid
  • Additional notes

    Protein concentration is above or equal to 0.05 µg/µl.

     This product was previously labelled as LEPRE1

     

  • 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

    • GROS 1
    • GROS1
    • Growth suppressor 1
    • LEPRE 1
    • Lepre1
    • Leprecan
    • Leprecan 1
    • Leprecan-1
    • Leprecan1
    • Leucine proline enriched proteoglycan (leprecan) 1
    • Leucine proline enriched proteoglycan 1
    • Leucine- and proline-enriched proteoglycan 1
    • MGC117314
    • OI8
    • P3H1
    • P3H1_HUMAN
    • Prolyl 3 hydroxylase 1
    • Prolyl 3-hydroxylase 1
    see all
  • Function

    Basement membrane-associated chondroitin sulfate proteoglycan (CSPG). Has prolyl 3-hydroxylase activity catalyzing the post-translational formation of 3-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens, especially types IV and V. May be involved in the secretory pathway of cells. Has growth suppressive activity in fibroblasts.
  • Involvement in disease

    Defects in LEPRE1 are the cause of osteogenesis imperfecta type 8 (OI8) [MIM:610915]. A connective tissue disorder characterized by disproportionate short stature, severe osteoporosis, shortening of the long bones, white sclerae, a round face and a short barrel-shaped chest.
  • Sequence similarities

    Belongs to the leprecan family.
    Contains 1 Fe2OG dioxygenase domain.
    Contains 4 TPR repeats.
  • Post-translational
    modifications

    O-glycosylated; chondroitin sulfate.
  • Cellular localization

    Endoplasmic reticulum. Secreted > extracellular space > extracellular matrix. Secreted into the extracellular matrix as a chondroitin sulfate proteoglycan.
  • Information by UniProt

Images

  • 12.5% SDS-PAGE analysis of ab132600 stained with Coomassie Blue.

References

ab132600 has not yet been referenced specifically in any publications.

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