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SHH Binds to the patched (PTC) receptor, which functions in association with smoothened (SMO), to activate the transcription of target genes. In the absence of SHH, PTC represses the constitutive signaling activity of SMO. Also regulates another target, the gli oncogene. Intercellular signal essential for a variety of patterning events during development: signal produced by the notochord that induces ventral cell fate in the neural tube and somites, and the polarizing signal for patterning of the anterior-posterior axis of the developing limb bud. Displays both floor plate- and motor neuron-inducing activity. The threshold concentration of N-product required for motor neuron induction is 5-fold lower than that required for floor plate induction. Interacts with HHATL/GUP1 which negatively regulates HHAT-mediated palmitoylation of the SHH N-terminus. N-product is active as a multimer. Expressed in fetal intestine, liver, lung, and kidney. Not expressed in adult tissues. Belongs to the hedgehog family. Note: This description may include information from UniProtKB.
Protein type: Cell cycle regulation; Cell development/differentiation; Motility/polarity/chemotaxis; Oncoprotein
Chromosomal Location of Human Ortholog: 7q36.3
Cellular Component: cell surface; cytosol; endoplasmic reticulum lumen; extracellular region; extracellular space; lipid raft; plasma membrane
Molecular Function: calcium ion binding; laminin-1 binding; morphogen activity; patched binding; protein binding; zinc ion binding
Biological Process: activation of hh target transcription factor; androgen metabolic process; axon guidance; branching morphogenesis of a tube; CD4-positive or CD8-positive, alpha-beta T cell lineage commitment; cell development; cell fate specification; cell-cell signaling; central nervous system development; dorsal/ventral pattern formation; embryonic development; embryonic digit morphogenesis; embryonic limb morphogenesis; embryonic pattern specification; forebrain development; granule cell precursor proliferation; heart development; heart looping; hindbrain development; lung development; lymphoid progenitor cell differentiation; male genitalia development; metanephros development; midbrain development; negative regulation of apoptosis; negative regulation of cell differentiation; negative regulation of cell migration; negative regulation of transcription from RNA polymerase II promoter; negative thymic T cell selection; neural crest cell migration; neuroblast proliferation; neuron fate commitment; pattern specification process; patterning of blood vessels; polarity specification of anterior/posterior axis; positive regulation of alpha-beta T cell differentiation; positive regulation of cell division; positive regulation of cell proliferation; positive regulation of immature T cell proliferation in the thymus; positive regulation of smoothened signaling pathway; positive regulation of T cell differentiation in the thymus; positive regulation of transcription from RNA polymerase II promoter; positive regulation of transcription, DNA-dependent; positive thymic T cell selection; prostate gland development; regulation of cell proliferation; regulation of odontogenesis; regulation of proteolysis; smoothened signaling pathway; stem cell development; T cell differentiation in the thymus; thymus development; ureteric bud branching; vasculogenesis; ventral midline development
Disease: Holoprosencephaly 3; Microphthalmia, Isolated, With Coloboma 5; Schizencephaly; Solitary Median Maxillary Central Incisor
Reference #:  Q15465 (UniProtKB)
Alt. Names/Synonyms: HHG-1; HHG1; HLP3; HPE3; MCOPCB5; SHH; SMMCI; sonic hedgehog homolog (Drosophila); Sonic hedgehog protein; Sonic hedgehog protein C-product; Sonic hedgehog protein N-product; TPT; TPTPS
Gene Symbols: SHH
Molecular weight: 49,607 Da
Basal Isoelectric point: 8.09  Predict pI for various phosphorylation states
CST Pathways:  Hedgehog Signaling
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
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