GLI3

Source: Wikipedia, the free encyclopedia.
GLI3
Available structures
Gene ontology
Molecular function
Cellular component
Biological process
Sources:Amigo / QuickGO
Ensembl
UniProt
RefSeq (mRNA)

NM_000168

NM_008130

RefSeq (protein)

NP_000159

NP_032156

Location (UCSC)Chr 7: 41.96 – 42.26 MbChr 13: 15.64 – 15.9 Mb
PubMed search[3][4]
Wikidata
View/Edit HumanView/Edit Mouse

Zinc finger protein GLI3 is a protein that in humans is encoded by the GLI3 gene.[5][6]

This gene encodes a

embryogenesis.[6]

Role in development

Gli3 is a known

Ci.[8] Gli3 mutant mice have many abnormalities including CNS and lung defects and limb polydactyly.[10][11][12][13][14] In the developing mouse limb bud, Gli3 derepression predominantly regulates Shh target genes.[15]

Disease association

Mutations in this gene have been associated with several diseases, including Greig cephalopolysyndactyly syndrome, Pallister–Hall syndrome, preaxial polydactyly type IV, and postaxial polydactyly types A1 and B.[6] DNA copy-number alterations that contribute to increased conversion of the oncogenes Gli1–3 into transcriptional activators by the Hedgehog signaling pathway are included in a genome-wide pattern, which was found to be correlated with an astrocytoma patient's outcome.[16][17]

There is evidence that the

dominant disorder Greig cephalopolysyndactyly syndrome (GCPS) that affects limb and craniofacial development in humans is caused by a translocations within the GLI3 gene.[18]

Interactions with Gli1 and Gli2

The independent

tumor BCC formation.[19]

Gli1 and Gli2 overexpression leads to BCC formation in mouse models and a one step model for tumour formation has been suggested in both cases. This also indicates that Gli1 and/or Gli2 overexpression is vital in BCC formation. Co-overexpression of Gli1 with Gli2 and Gli2 with Gli3 leads to transgenic mice malformations and death, respectively, but not the formation of BCC. This suggests that overexpression of more than one Gli protein is not necessary for BCC formation.

Interactions

GLI3 has been shown to

interact with CREBBP[20] SUFU,[21] ZIC1,[22] and ZIC2.[22]

References

  1. ^ a b c GRCh38: Ensembl release 89: ENSG00000106571Ensembl, May 2017
  2. ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000021318Ensembl, May 2017
  3. ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. PMID 2118997
    .
  6. ^ a b c "Entrez Gene: GLI3 GLI-Kruppel family member GLI3 (Greig cephalopolysyndactyly syndrome)".
  7. S2CID 4414768
    .
  8. ^ .
  9. .
  10. .
  11. .
  12. .
  13. .
  14. .
  15. .
  16. .
  17. .
  18. .
  19. .
  20. .
  21. .
  22. ^ .

External links

This article incorporates text from the United States National Library of Medicine, which is in the public domain.

This page is based on the copyrighted Wikipedia article: GLI3. Articles is available under the CC BY-SA 3.0 license; additional terms may apply.Privacy Policy