Anti-Ihh Antibody (RMAB-0250742)
Cat. No.: RMAB-0250742
Category: Primary Antibodies
INQUIRY
100 μL
Customer Size
Rabbit monoclonal to Ihh
Product Features
| Isotype | IgG |
|---|---|
| Clonality | Monoclonal |
| Host Species | Rabbit |
| Clone Number | EP1192Y |
| Form | Liquid |
| Purity | Protein A purified |
| Species Reactivity | Human, Mouse, Rat |
| Immunogen | Synthetic peptide. |
| Applications | WB, IHC-P |
| Key Features | Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply; Rabbit monoclonal to Ihh; Suitable for: WB, IHC-P; Reacts with: Mouse, Rat, Human |
Target Information
| Target Symbol | IHH |
|---|---|
| Target Name | Indian hedgehog protein |
| UniProt ID | Q14623 |
| 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. |
| 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). |
| Involvement in Disease | Defects in IHH are the cause of brachydactyly type A1 (BDA1). 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). 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. |
| 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. |
| Sequence Similarities | Belongs to the hedgehog family. |
Storage & Shipping
| Storage Buffer | pH: 7.20; Preservative: 0.01% Sodium azide; Constituents: 59% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA |
|---|---|
| Storage & Shipping | Shipped at 4°C. Store at 4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. |
For research use only. Not for clinical use.
