Name: U-Blot® Ihh Rabbit mAb
SKU: WR5036
Price: 268 USD
Availability: InStock

Product details

Background:This gene encodes a member of the hedgehog family of proteins. The encoded preproprotein is proteolytically processed to generate multiple protein products, including an N-terminal fragment that is involved in signaling. Hedgehog family proteins are essential secreted signaling molecules that regulate a variety of developmental processes including growth, patterning and morphogenesis. The protein encoded by this gene specifically plays a role in bone growth and differentiation. Mutations in this gene are the cause of brachydactyly type A1, which is characterized by shortening or malformation of the fingers and toes. Mutations in this gene are also the cause of acrocapitofemoral dysplasia. [provided by RefSeq, Nov 2015],

Specifications

Size	50μL/100μL
Target	Ihh
Reactivity	Human, Mouse, Rat
Applications	WB, IHC, IF, ELISA
MW(Calculated)	45kD
MW(Observed)	45kD
Host Species	Rabbit
Isotype	IgG,Kappa
Conjugate/Modification	Unmodified
Recommended Dilution Ratio	IHC 1:200-1:1000; WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004)
Formulation	PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
Purification	Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography.
Storage	-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
Clonality	Monoclonal
Clone Number	RM1268R
Immunogen	The specific immunogen used to produce this antibody is proprietary information.
Specificity	Endogenous
Gene Name	IHH
Protein Name	Indian hedgehog protein
Other Name	IHH; / Indian hedgehog protein; / IHH; / HHG-2
Species	Human
Gene ID	3549
Uniprot	Q14623,
Species.1	Mouse
Gene ID.1	16147
Uniprot.1	P97812
Species.2
Gene ID.2
Uniprot.2
Cellular Localization	[Indian hedgehog protein N-product]: Cell membrane; Lipid-anchor; Extracellular side . The N-terminal peptide remains associated with the cell surface. .; [Indian hedgehog protein C-product]: Secreted, extracellular space . The C-terminal peptide diffuses from the cell. .; Cell membrane .
Tissue Expression	Expressed in embryonic lung, and in adult kidney and liver.
Signaling_pathway	Environmental Information Processing >> Signal transduction >> Hedgehog signaling pathway
Research Areas	>>Hedgehog signaling pathway; / >>Proteoglycans in cancer
Function	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.,