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Proteins/UNIPROT:O43323
[Desert hedgehog protein]: Precursor of desert hedgehog, a morphogen that activates the smoothened signaling pathway, and which is essential for a variety of patterning events during development (By similarity). The C-terminal part of the precursor displays an autoproteolysis and a cholesterol transferase activity, resulting (1) in the cleavage of the full-length protein into two parts, desert hedgehog protein N-product and C-product and (2) covalent attachment of a cholesterol moiety to the C-terminus of the newly generated N-product (DHH-N) (By similarity). Both autoproteolysis and a cholesterol transferase activities occur in the endoplasmic reticulum (By similarity). Following additional lipidation, DHH-N acts as a morphogen, while the C-product is degraded in the endoplasmic reticulum (By similarity)..
[Desert hedgehog protein N-product]: The dually lipidated desert hedgehog protein N-product is a morphogen that activates the smoothened signaling pathway, and which is essential for a variety of patterning events during development (By similarity). Acts by binding to the patched receptor (PTCH1 or PTCH2), relieving smoothened (SMO) inhibition by patched, activating the smoothened signaling pathway and transcription of target genes. In the absence of DHH, patched represses the constitutive signaling activity of SMO (By similarity). DHH plays a key role in male gonad development (spermatogenesis) and the formation of Schwann cells (By similarity). Required for normal testis development and spermatogenesis, namely for the formation of adult-type Leydig cells and normal development of peritubular cells and seminiferous tubules (By similarity). In Schwann cells, controls the development of the peripheral nerve sheath and the transition of mesenchymal cells to form the epithelium-like structure of the perineurial tube (By similarity). May induce motor neurons in lateral neural tube and may have a polarizing activity. Involved in tissue regeneration: secreted from epithelial neuroendocrine cells and elicits a regenerative response from mesenchymal cells (By similarity). Functions in cell-cell mediated juxtacrine signaling. Activates primary cilia signaling on neighboring valve interstitial cells through a paracrine mechanism (By similarity). Promotes endothelium integrity..
Function from UniProtKB (O43323), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
2 rows
74 statements
74 statements · 5 reference databases · Subject: DHH · Sources: gene ontology, Hetionet, Reactome, Rhea, WikiPathways
1 reaction
glycyl-L-cysteinyl-[protein]+cholesterol+hydron[protein]-C-terminal glycyl cholesterol ester+N-terminal L-cysteinyl-[protein]
Catalysed by DHH or IHH or SHH
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:59504CC BY 4.0Reference data
3 names
Resolves to: DHH