Rat Anti-IHH Recombinant Antibody (9L16) (CBMAB-I1847-YY)

Rat

Human

9L16

IgG2

WB, ELISA

Human

IgG2

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Liquid

LYOPH

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Indian hedgehog homolog (Drosophila)

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.

Indian Hedgehog

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) (By similarity).

Bone resorption Source: Ensembl
Branching involved in blood vessel morphogenesis Source: Ensembl
Camera-type eye photoreceptor cell fate commitment Source: Ensembl
Cartilage development Source: AgBase
Cell-cell signaling Source: InterPro
Cell fate specification Source: GO_Central
Cell maturation Source: Ensembl
Chondrocyte differentiation involved in endochondral bone morphogenesis Source: Ensembl
Chondrocyte proliferation Source: Ensembl
Embryonic camera-type eye morphogenesis Source: Ensembl
Embryonic digestive tract morphogenesis Source: Ensembl
Embryonic digit morphogenesis Source: AgBase
Embryonic pattern specification Source: Ensembl
Embryonic skeletal joint development Source: Ensembl
Epithelial cell-cell adhesion Source: Ensembl
Epithelial cell morphogenesis Source: Ensembl
Head morphogenesis Source: Ensembl
Heart looping Source: BHF-UCL
Intein-mediated protein splicing Source: InterPro
In utero embryonic development Source: Ensembl
Liver regeneration Source: Ensembl
Maternal process involved in female pregnancy Source: Ensembl
Multicellular organism growth Source: Ensembl
Negative regulation of alpha-beta T cell differentiation Source: BHF-UCL
Negative regulation of apoptotic process Source: Ensembl
Negative regulation of eye pigmentation Source: Ensembl
Negative regulation of immature T cell proliferation in thymus Source: BHF-UCL
Negative regulation of T cell differentiation in thymus Source: BHF-UCL
Neuron development Source: Ensembl
Osteoblast differentiation Source: Ensembl
Pancreas development Source: Ensembl
Positive regulation of alpha-beta T cell differentiation Source: BHF-UCL
Positive regulation of collagen biosynthetic process Source: Ensembl
Positive regulation of epithelial cell proliferation Source: Ensembl
Positive regulation of mesenchymal cell proliferation Source: Ensembl
Positive regulation of smoothened signaling pathway Source: BHF-UCL
Positive regulation of T cell differentiation in thymus Source: BHF-UCL
Positive regulation of transcription by RNA polymerase II Source: Ensembl
Protein autoprocessing Source: InterPro
Proteoglycan metabolic process Source: Ensembl
Regulation of gene expression Source: GO_Central
Regulation of growth Source: Ensembl
Response to estradiol Source: Ensembl
Response to mechanical stimulus Source: Ensembl
Retinal pigment epithelium development Source: Ensembl
Skeletal system development Source: UniProtKB
Smoothened signaling pathway Source: UniProtKB
Smooth muscle tissue development Source: Ensembl
Somite development Source: BHF-UCL
Vitelline membrane formation Source: Ensembl

Indian hedgehog protein N-product: Cell membrane. The N-terminal peptide remains associated with the cell surface.
Indian hedgehog protein C-product: Extracellular space. The C-terminal peptide diffuses from the cell. Cell membrane

Brachydactyly A1 (BDA1):
A form of brachydactyly. Brachydactyly defines a group of inherited malformations characterized by shortening of the digits due to abnormal development of the phalanges and/or the metacarpals. Brachydactyly type A1 is 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. BDA1 inheritance is autosomal dominant.
Acrocapitofemoral dysplasia (ACFD):
An autosomal recessive 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 are also present to a variable extent at the shoulders, knees and ankles.

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 (By similarity).
Cholesterylation is required for N-product targeting to lipid rafts and multimerization.
Palmitoylated. N-palmitoylation is required for N-product multimerization and full activity.