Desert hedgehog (protein)

2018-03-16T03:26:37Z

2601:648:8200:C7B0:A9F4:D89F:8F68:78BD:

{{Infobox_gene}}
{{infobox protein
|Name=desert hedgehog
|caption=
|image=
|width=
|HGNCid=2865
|Symbol=DHH
|AltSymbols=HHG-3, MGC35145
|EntrezGene=50846
|OMIM=605423
|RefSeq=NM_021044
|UniProt= O43323
|PDB=
|ECnumber=
|Chromosome=12
|Arm=q
|Band=13.1
|LocusSupplementaryData=
}}
'''Desert hedgehog''', also '''Desert hedgehog homolog''' or '''Dhh''', is a protein encoded by the '''DHH''' gene, and is a member of the [[hedgehog signaling pathway]]. The human homolog (DHH) is on [[chromosome]] band 12q13.1.<ref name="pmid10640830">{{cite journal | vauthors = Kamisago M, Kimura M, Furutani Y, Furutani M, Takao A, Momma K, Matsuoka R | title = Assignment of human desert hedgehog gene (DHH) to chromosome band 12q13.1 by in situ hybridization | journal = Cytogenet. Cell Genet. | volume = 87 | issue = 1–2 | pages = 117–8 | year = 1999 | pmid = 10640830 | doi = 10.1159/000015376 | url = }}</ref><ref name="pmid10773676">{{cite journal | vauthors = Tate G, Satoh H, Endo Y, Mitsuya T | title = Assignment of desert hedgehog (DHH) to human chromosome bands 12q12→q13.1 by in situ hybridization | journal = Cytogenet. Cell Genet. | volume = 88 | issue = 1–2 | pages = 93–4 | year = 2000 | pmid = 10773676 | doi = 10.1159/000015495 | url = }}</ref> The protein encoded by this gene is involved in [[cell signaling]]. The several mammalian variants of the ''[[Drosophila]]'' hedgehog gene ([[hedgehog signaling pathway#Discovery|which was the first named]]) have been named after the various species of [[hedgehog]]; the [[desert hedgehog]] is honored by this one. The gene is not specific to desert hedgehogs.

== Function ==
This gene encodes a member of the hedgehog family. The hedgehog gene family encodes signaling molecules that play an important role in regulating [[morphogenesis]]. This protein is predicted to be made as a precursor that is [[autocatalysis|autocatalytically]] cleaved; the [[N-terminus|N-terminal]] portion is soluble and contains the signalling activity while the [[C-terminus|C-terminal]] portion is involved in precursor processing. More importantly, the C-terminal product [[covalently]] attaches a [[cholesterol]] [[moiety (chemistry)|moiety]] to the N-terminal product, restricting the N-terminal product to the cell surface and preventing it from freely [[diffusing]] throughout the organism. Defects in this protein have been associated with partial [[gonadal dysgenesis]] (PGD) accompanied by minifascicular [[polyneuropathy]]. This protein may be involved in both male gonadal [[Differentiation (cellular)|differentiation]] and [[perineurium|perineurial]] development.<ref name="entrez">{{cite web | title = Entrez Gene: desert hedgehog homolog (Drosophila)| url = https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=50846| accessdate = }}</ref>

== Clinical Significance ==
Defects in this protein have been associated with partial [[XY gonadal dysgenesis|gonadal dysgenesis]] (PGD) accompanied by minifascicular [[polyneuropathy]]. This protein may be involved in both male [[gonad]]al differentiation and [[perineurium|perineurial]] development.<ref name="entrez"/>

==References==
{{Reflist}}

==External links==
* {{MeshName|DHH+protein,+human|3=DHH protein, human}}

{{Hedgehog signaling pathway}}

{{NLM content}}

{{gene-12-stub}}

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Desert hedgehog (protein)