Hedgehog signaling pathway

Protein hedgehog
Identifiers
OrganismDrosophila melanogaster
Symbolhh
UniProtQ02936
Search for
StructuresSwiss-model
DomainsInterPro
Hedge N-terminal signal
Identifiers
SymbolHH_signal
PfamPF01085
InterProIPR000320
CATH3d1m
SCOP23d1m / SCOPe / SUPFAM
Available protein structures:
Pfam  structures / ECOD  
PDBRCSB PDB; PDBe; PDBj
PDBsumstructure summary
See also InterProIPR001657.

The Hedgehog signaling pathway is a signaling pathway that transmits information to embryonic cells required for proper cell differentiation. Different parts of the embryo have different concentrations of hedgehog signaling proteins. The pathway also has roles in the adult. Diseases associated with the malfunction of this pathway include cancer.[1][2]

The Hedgehog signaling pathway is one of the key regulators of animal development and is present in all bilaterians.[3] The pathway takes its name from its polypeptide ligand, an intracellular signaling molecule called Hedgehog (Hh) found in fruit flies of the genus Drosophila; fruit fly larvae lacking the Hh gene are said to resemble hedgehogs. Hh is one of Drosophila's segment polarity gene products, involved in establishing the basis of the fly body plan. The molecule remains important during later stages of embryogenesis and metamorphosis.

Mammals have three Hedgehog homologues, Desert (DHH), Indian (IHH), and Sonic (SHH), of which Sonic is the best studied. The pathway is equally important during vertebrate embryonic development and is therefore of interest in evolutionary developmental biology. In knockout mice lacking components of the pathway, the brain, skeleton, musculature, gastrointestinal tract and lungs fail to develop correctly. Recent studies point to the role of Hedgehog signaling in regulating adult stem cells involved in maintenance and regeneration of adult tissues. The pathway has also been implicated in the development of some cancers.[1] Drugs that specifically target Hedgehog signaling to fight this disease are being actively developed by a number of pharmaceutical companies.

  1. ^ a b Jamieson C, Martinelli G, Papayannidis C, Cortes JE (September 2020). "Hedgehog Pathway Inhibitors: A New Therapeutic Class for the Treatment of Acute Myeloid Leukemia". Blood Cancer Discovery. 1 (2): 134–145. doi:10.1158/2643-3230.BCD-20-0007. PMC 8447269. PMID 34661144.
  2. ^ Kimball JW (18 February 2008). "The Hedgehog Signaling Pathway". Kimball's Biology Pages. Archived from the original on 26 June 2008.
  3. ^ Ingham PW, Nakano Y, Seger C (June 2011). "Mechanisms and functions of Hedgehog signalling across the metazoa". Nature Reviews. Genetics. 12 (6): 393–406. doi:10.1038/nrg2984. PMID 21502959. S2CID 33769324.