Sonic hedgehog protein

SHH
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 3HO5, 3M1N, 3MXW
Identifiers
Aliases	SHH, HHG1, HLP3, HPE3, MCOPCB5, SMMCI, TPT, TPTPS, sonic hedgehog, Sonic hedgehog, ShhNC, sonic hedgehog signaling molecule
External IDs	OMIM: 600725; MGI: 98297; HomoloGene: 30961; GeneCards: SHH; OMA:SHH - orthologs
Gene location (Human) Chr. Chromosome 7 (human)[1] Band 7q36.3 Start 155,799,980 bp[1] End 155,812,463 bp[1]
Gene location (Mouse) Chr. Chromosome 5 (mouse)[2] Band 5 B1|5 14.39 cM Start 28,661,813 bp[2] End 28,672,254 bp[2]
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in right lobe of liver pancreatic epithelial cell sural nerve right adrenal cortex left adrenal cortex testicle mucosa of transverse colon gallbladder urinary bladder spinal ganglia Top expressed in transitional epithelium of urinary bladder tooth molar incisor oral mucosa tongue Hindgut left lung lobe notochord epithelium of stomach More reference expression data BioGPS More reference expression data
Gene ontology Molecular function calcium ion binding metal ion binding patched binding peptidase activity zinc ion binding laminin-1 binding glycosaminoglycan binding morphogen activity hydrolase activity protein binding endopeptidase activity Cellular component cytosol endoplasmic reticulum lumen membrane cell surface membrane raft plasma membrane extracellular space extracellular region extracellular matrix Biological process positive regulation of skeletal muscle cell proliferation pattern specification process myotube differentiation mesenchymal cell proliferation male genitalia development bud outgrowth involved in lung branching limb bud formation T cell differentiation in thymus embryonic pattern specification positive regulation of skeletal muscle tissue development positive regulation of immature T cell proliferation in thymus positive regulation of kidney smooth muscle cell differentiation negative regulation of Wnt signaling pathway anatomical structure formation involved in morphogenesis ectoderm development hindgut morphogenesis spinal cord dorsal/ventral patterning formation of anatomical boundary oligodendrocyte differentiation trachea development prostate gland development negative regulation of transcription elongation from RNA polymerase II promoter limb development vasculogenesis primary prostatic bud elongation heart looping telencephalon regionalization odontogenesis of dentin-containing tooth positive regulation of mesenchymal cell proliferation angiogenesis positive regulation of epithelial cell proliferation involved in prostate gland development positive regulation of striated muscle cell differentiation negative regulation of proteasomal ubiquitin-dependent protein catabolic process embryonic digestive tract morphogenesis apoptotic signaling pathway inner ear development hair follicle morphogenesis cell population proliferation metanephros development lung epithelium development negative regulation of canonical Wnt signaling pathway regulation of proteolysis branching involved in salivary gland morphogenesis negative regulation of mesenchymal cell apoptotic process positive regulation of neuroblast proliferation dopaminergic neuron differentiation intermediate filament organization renal system development cell fate commitment regulation of transcription, DNA-templated positive regulation of smoothened signaling pathway androgen metabolic process kidney development lung development negative regulation of protein catabolic process thymus development embryonic organ development negative regulation of cell differentiation negative regulation of dopaminergic neuron differentiation embryonic digit morphogenesis negative regulation of alpha-beta T cell differentiation positive regulation of sclerotome development epithelial-mesenchymal cell signaling negative regulation of gene expression lymphoid progenitor cell differentiation negative thymic T cell selection positive regulation of transcription, DNA-templated positive regulation of Wnt signaling pathway ventral midline development central nervous system development heart development branching involved in ureteric bud morphogenesis negative regulation of T cell proliferation embryonic limb morphogenesis positive regulation of cell differentiation branching involved in prostate gland morphogenesis positive regulation of mesenchymal cell proliferation involved in ureter development establishment of cell polarity branching involved in blood vessel morphogenesis positive regulation of cerebellar granule cell precursor proliferation neuroblast proliferation pancreas development positive regulation of protein import into nucleus smoothened signaling pathway oligodendrocyte development camera-type eye development spinal cord motor neuron differentiation positive regulation of alpha-beta T cell differentiation digestive tract morphogenesis hair follicle development positive regulation of oligodendrocyte differentiation roof of mouth development left lung development striated muscle tissue development positive regulation of T cell differentiation in thymus cellular response to lithium ion embryonic foregut morphogenesis anatomical structure development thalamus development stem cell development smoothened signaling pathway involved in regulation of cerebellar granule cell precursor cell proliferation cell fate specification negative regulation of apoptotic process negative regulation of transcription by RNA polymerase II regulation of nodal signaling pathway involved in determination of lateral mesoderm left/right asymmetry proteolysis embryonic skeletal system development regulation of protein localization to nucleus odontogenesis respiratory tube development regulation of prostatic bud formation thyroid gland development negative regulation of cell migration developmental growth right lung development determination of left/right asymmetry in lateral mesoderm positive thymic T cell selection mesenchymal smoothened signaling pathway involved in prostate gland development canonical Wnt signaling pathway regulation of gene expression osteoblast development negative regulation of cholesterol efflux dorsal/ventral neural tube patterning regulation of epithelial cell proliferation involved in prostate gland development epithelial-mesenchymal signaling involved in prostate gland development dorsal/ventral pattern formation branching morphogenesis of an epithelial tube lung lobe morphogenesis embryonic forelimb morphogenesis midbrain development skin development regulation of odontogenesis regulation of mesenchymal cell proliferation involved in prostate gland development mesenchymal cell proliferation involved in lung development positive regulation of hh target transcription factor activity prostate epithelial cord elongation metanephric mesenchymal cell proliferation involved in metanephros development endocytosis lung morphogenesis cell development trachea morphogenesis epithelial cell proliferation involved in salivary gland morphogenesis cell-cell signaling salivary gland cavitation hindbrain development blood coagulation negative regulation of kidney smooth muscle cell differentiation somite development cerebellar granule cell precursor proliferation axon guidance negative regulation of ureter smooth muscle cell differentiation positive regulation of ureter smooth muscle cell differentiation multicellular organism development animal organ formation intein-mediated protein splicing determination of left/right symmetry vasculature development positive regulation of gene expression regulation of cell population proliferation embryonic morphogenesis neural crest cell migration polarity specification of anterior/posterior axis positive regulation of cell population proliferation artery development protein localization to nucleus Bergmann glial cell differentiation forebrain development neuron fate commitment CD4-positive or CD8-positive, alpha-beta T cell lineage commitment striated muscle cell differentiation myoblast differentiation lung-associated mesenchyme development epithelial tube branching involved in lung morphogenesis positive regulation of cell division positive regulation of transcription by RNA polymerase II anterior/posterior pattern specification embryonic hindlimb morphogenesis forebrain regionalization cell proliferation in external granule layer tracheoesophageal septum formation regulation of signaling receptor activity Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 6469 20423 Ensembl ENSG00000164690 ENSMUSG00000002633 UniProt Q15465 Q62226 RefSeq (mRNA) NM_000193 NM_001310462 NM_009170 RefSeq (protein) NP_000184 NP_001297391 NP_033196 Location (UCSC) Chr 7: 155.8 – 155.81 Mb Chr 5: 28.66 – 28.67 Mb PubMed search [3] [4]
Wikidata
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Sonic hedgehog protein (SHH) is encoded for by the SHH gene.^[5] The protein is named after the video game character Sonic the Hedgehog.

This signaling molecule is key in regulating embryonic morphogenesis in all animals. SHH controls organogenesis and the organization of the central nervous system, limbs, digits and many other parts of the body. Sonic hedgehog is a morphogen that patterns the developing embryo using a concentration gradient characterized by the French flag model.^[6] This model has a non-uniform distribution of SHH molecules which governs different cell fates according to concentration. Mutations in this gene can cause holoprosencephaly, a failure of splitting in the cerebral hemispheres,^[7] as demonstrated in an experiment using SHH knock-out mice in which the forebrain midline failed to develop and instead only a single fused telencephalic vesicle resulted.^[8]

Sonic hedgehog still plays a role in differentiation, proliferation, and maintenance of adult tissues. Abnormal activation of SHH signaling in adult tissues has been implicated in various types of cancers including breast, skin, brain, liver, gallbladder and many more.^[9]