GLI3
[GLI3 Protein is a putative zinc finger DNA-binding transcription factor of the GLI-Kruppel family. GLI3 is homologous to Drosophila Cubitus interruptus (Ci), which regulates patched, gooseberry, and decapentaplegic genes. In response to hedgehog signaling, the full-length cytoplasmic transcriptional activator form of Ci can be cleaved to a nuclear transcription repressor form. Based on GLI3 mutations and phenotypes, GLI3 shows transcriptional activation or repression, and subcellular localization, similar to Ci. Cytoplasmic GLI3 activates PTCH gene expression. PKA-dependent processing of vertebrate GLI3 in the developing limb generates the repressor form, which appears to be antagonized by sonic hedgehog signaling. Mutations of GLI3 may be responsible for Greig Cephalopolysyndactyly Syndrome, Pallister-Hall Syndrome, and Postaxial Polydactyly, suggesting specific roles during development. (from OMIM 165240 and NCI) ( NCI )]
UMLS (NCI) C0082708- Amino Acid, Peptide, or Protein
- Biologically Active Substance
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