Recombinant human Ihh protein (Active) (ab243268)
Key features and details
- Expression system: Escherichia coli
- Endotoxin level: < 1.000 Eu/µg
- Active: Yes
- Suitable for: SDS-PAGE, Functional Studies, HPLC
Description
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Product name
Recombinant human Ihh protein (Active)
See all Ihh proteins and peptides -
Biological activity
Fully biologically active when compared to standard. The ED50 as determined by its ability to induce alkaline phosphatase production by C3H10T1/2(CCL-226) cells is 3.0-10 μg/ml.
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Endotoxin level
< 1.000 Eu/µg -
Expression system
Escherichia coli -
Accession
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Protein length
Protein fragment -
Animal free
No -
Nature
Recombinant -
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Species
Human -
Sequence
IIGPGRVVGSRRRPPRKLVPLAYKQFSPNVPEKTLGASGRYEGKIARSSE RFKELTPNYNPDIIFKDEENTGADRLMTQRCKDRLNSLAISVMNQWPGVK LRVTEGWDEDGHHSEESLHYEGRAVDITTSDRDRNKYGLLARLAVEAGFD WVYYESKAHVHCSVKSEHSAAAKTGG -
Predicted molecular weight
20 kDa -
Amino acids
29 to 202
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Specifications
Our Abpromise guarantee covers the use of ab243268 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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Applications
SDS-PAGE
Functional Studies
HPLC
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Form
Lyophilized -
Concentration information loading...
Preparation and Storage
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Stability and Storage
Shipped at 4°C. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
Constituents: Phosphate Buffer, 0.87% Sodium chloride
0.2 µm filteredThis product is an active protein and may elicit a biological response in vivo, handle with caution.
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ReconstitutionWe recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Reconstitute in sterile distilled water or aqueous buffer containing 0.1% BSA to a concentration of 0.1-1.0 mg/mL. Stock solutions should be apportioned into working aliquots and stored at =-20 °C. Further dilutions should be made in appropriate buffered solutions.
General Info
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Alternative names
- BDA1
- HHG-2
- HHG2
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
modificationsThe 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
Protocols
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
Datasheets and documents
References (1)
ab243268 has been referenced in 1 publication.
- Tarulli GA et al. Discrete Hedgehog Factor Expression and Action in the Developing Phallus. Int J Mol Sci 21:N/A (2020). PubMed: 32059607