Zinc finger protein GLI3 is a protein that in humans is encoded by the GLI3 gene. [5][6] This gene encodes a protein that belongs to the C2H2-type zinc finger proteins subclass of the Gli family. They are characterized as DNA-binding transcription factors and are mediators of Sonic hedgehog (Shh) signaling.

May 25, 2000 · GLI3-related Pallister-Hall syndrome (GLI3-PHS) is characterized by a spectrum of anomalies ranging from polydactyly, asymptomatic bifid epiglottis, and hypothalamic hamartoma at the mild end to laryngotracheal cleft with neonatal lethality at the severe end.

The transcription factor GLI3 is a member of the Hedgehog (Hh/HH) signaling pathway that can exist as a full length (Gli3-FL/GLI3-FL) or repressor (Gli3-R/GLI3-R) form. In response to HH activation, GLI3-FL regulates HH genes by targeting the GLI1 promoter.

The GLI3 gene belongs to a family of genes that are involved in the normal shaping (patterning) of many tissues and organs during the early stages of development before birth. Learn about this gene and related health conditions.

Mar 30, 2025 · GLI3 (GLI Family Zinc Finger 3) is a Protein Coding gene. Diseases associated with GLI3 include Greig Cephalopolysyndactyly Syndrome and Polydactyly, Postaxial, Type A1. Among its related pathways are Gene expression (Transcription) and Signaling by Hedgehog.

GLI3 encodes a zinc finger transcription factor that functions in the hedgehog (Hh; see SHH, 600725) signal transduction pathway, which is dependent on primary cilia in many tissues. GLI3 undergoes posttranslational maturation into 2 isoforms with antagonistic transcriptional activities.

Apr 3, 2020 · The absence of Gli3 in mice impaired brain and lung development and GLI3 mutations in humans are the cause of Greig cephalopolysyndactyly (GCPS) and Pallister Hall syndromes (PHS). In the immune system GLI3 regulates B, T and NK-cells and may be involved in LPS-TLR4 signaling.

Gli proteins are members of the family of Kruppel-like factors with highly conserved Zn finger DNA-binding domains. In mammals, they are represented by three proteins: Gli1, Gli2 and Gli3. Gli1 acts principally as a transcriptional activator, whereas Gli2 and Gli3 display both activator and repressor functions [15, 16, 17].

Feb 19, 2025 · Title: GLI3 Is Stabilized by SPOP Mutations and Promotes Castration Resistance via Functional Cooperation with Androgen Receptor in Prostate Cancer. Two nonsense GLI3 variants are associated with polydactyly and syndactyly in two families by affecting the sonic hedgehog signaling pathway.

GLI3 Is Stabilized by SPOP Mutations and Promotes Castration Resistance via Functional Cooperation with Androgen Receptor in Prostate Cancer. Two nonsense GLI3 variants are associated with polydactyly and syndactyly in two families by affecting the sonic hedgehog signaling pathway.