- Target
- Ihh
- Reactivity
- Human,Mouse,Rat
- Application
- WB,IHC,IF,ELISA
- MW(Calculated)
- 45kD
- MW(Observed)
- 45kD
- Host Species
- Rabbit
- Isotype
- IgG,Kappa
- Conjugate/Modification
- Unmodified
- Modification site
- --
- Recommended Dilution Ratio
- IHC 1:200-1:1000;WB 1:2000-1:10000;IF 1:200-1:1000;ELISA 1:5000-1:20000;
- Form
- PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
- Source
- --
- Purification
- Protein A
- Purity
- --
- storageCondition
- -15°C to -25°C/1 year(Do not lower than -25°C)
- Concentration
- --
- Clonality
- Monoclonal
- Clone No.
- PT1268R
- Immunogen
- --
- Sequence
- --
- Specificity
- Endogenous
- Gene Name
- IHH
- Protein Name
- Indian hedgehog protein
- Other Name
- IHH;Indian hedgehog protein;IHH;HHG-2
- Organism-1
- Human
- Gene ID-1
- 3549
- SwissProt-1
- Q14623
- Organism-2
- Mouse
- Gene ID-2
- 16147
- SwissProt-2
- P97812
- Organism-3
- --
- Gene ID-3
- --
- SwissProt-3
- --
- Organism-4
- --
- gene ID-4
- --
- SwissProt-4
- --
- Cellular Localization
- Disease: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.,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.,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).,PTM:Cholesterylation is required for N-product targeting to lipid rafts and multimerization.,PTM:Palmitoylated. N-palmitoylation is required for N-product multimerization and full activity.,PTM: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.,similarity:Belongs to the hedgehog family.,subcellular location:The C-terminal peptide diffuses from the cell.,subcellular location:The N-terminal peptide remains associated with the cell surface.,tissue specificity:Expressed in embryonic lung, and in adult kidney and liver.,