Overview

  • Product name

    Recombinant human Ihh protein (Active) (Animal Free)
    See all Ihh proteins and peptides
  • Protein length

    Full length protein
  • Description

    Recombinant human Ihh protein (Active)

Description

  • Nature

    Recombinant
  • Source

    Escherichia coli
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Sequence

      MIIGPGRVVG SRRRPPRKLV PLAYKQFSPN VPEKTLGASG RYEGKIARSS ERFKELTPNY NPDIIFKDEE NTGADRLMTQ RCKDRLNSLA ISVMNQWPGV KLRVTEGWDE DGHHSEESLH YEGRAVDITT SDRDRNKYGL LARLAVEAGF DWVYYESKAH VHCSVKSEHS AAAKTGG
    • Molecular weight

      20 kDa
    • Amino acids

      29 to 202
    • Additional sequence information

      This product is for the mature full length N product protein. The signal peptide is not included.

Specifications

Our Abpromise guarantee covers the use of ab218097 in the following tested applications.

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

  • Biological activity

    The activity is determined by the dose-dependent induction of alkaline phosphatase activity of CCL-226 cells and is typically less than 10 μg/mL. This corresponds to an expected specific activity of 1 x 102 units/mg.

  • Applications

    Functional Studies

    SDS-PAGE

  • Endotoxin level

    < 0.050 Eu/µg
  • Purity

    > 97 % SDS-PAGE.
    Purity is typically 97% as determined by reducing and non-reducing SDS-PAGE.
  • Form

    Lyophilised
  • Additional notes

    This product shares 100% homology with amino acids 29 -202 of Mouse IHH.

  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at -20°C or -80°C.

    This product is an active protein and may elicit a biological response in vivo, handle with caution.

  • Reconstitution
    Reconstitute in sterile 10 mM HCl at 0.1 mg/mL. Centrifuge vial before opening. Suspend the product by gently pipetting the above recommended solution down the sides of the vial. DO NOT VORTEX. Allow several minutes for complete reconstitution. For prolonged storage, dilute to working aliquots in a 0.1% BSA solution, store at -80 ºC and avoid repeat freeze thaws.

General Info

  • Alternative names

    • BDA1
    • HHG-2
    • HHG2
    • IHH
    • IHH_HUMAN
    • Indian Hedgehog
    • Indian hedgehog homolog
    • Indian hedgehog protein
    • Indian hedgehog protein C-product
    • Indian hedgehog protein N-product
    see all
  • Function

    Intercellular signal essential for a variety of patterning events during development. Binds to the patched (PTC) receptor, which functions in association with smoothened (SMO), to activate the transcription of target genes. Implicated in endochondral ossification: may regulate the balance between growth and ossification of the developing bones. Induces the expression of parathyroid hormone-related protein (PTHRP).
  • Tissue specificity

    Expressed in embryonic lung, and in adult kidney and liver.
  • Involvement in disease

    Defects in IHH are the cause of brachydactyly type A1 (BDA1) [MIM:112500]. BDA1 is an autosomal dominant disorder characterized by middle phalanges of all the digits rudimentary or fused with the terminal phalanges. The proximal phalanges of the thumbs and big toes are short.
    Defects in IHH are a cause of acrocapitofemoral dysplasia (ACFD) [MIM:607778]. ACFD is a disorder characterized by short stature of variable severity with postnatal onset. The most constant radiographic abnormalities are observed in the tubular bones of the hands and in the proximal part of the femur. Cone-shaped epiphyses or a similar epiphyseal configuration with premature epimetaphyseal fusion result in shortening of the skeletal components involved. Cone-shaped epiphyses were also present to a variable extent at the shoulders, knees, and ankles.
  • Sequence similarities

    Belongs to the hedgehog family.
  • Post-translational
    modifications

    The C-terminal domain displays an autoproteolysis activity and a cholesterol transferase activity. Both activities result in the cleavage of the full-length protein and covalent attachment of a cholesterol moiety to the C-terminal of the newly generated N-terminal fragment (N-product). The N-product is the active species in both local and long-range signaling, whereas the C-product has no signaling activity.
    Cholesterylation is required for N-product targeting to lipid rafts and multimerization.
    Palmitoylated. N-palmitoylation is required for N-product multimerization and full activity.
  • Cellular localization

    Secreted > extracellular space. The C-terminal peptide diffuses from the cell and Cell membrane. The N-terminal peptide remains associated with the cell surface.
  • Information by UniProt

References

ab218097 has not yet been referenced specifically in any publications.

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