Target intelligence / Profile preview

GLIS family zinc finger 3 (GLIS3)

Target
GLIS3
Molecular classification
Transcription factor, Zinc finger protein, Krüppel-like zinc finger family
01

Overview

GLIS family zinc finger 3 (GLIS3) is a Krüppel-like zinc finger transcription factor containing five C2H2-type zinc finger domains. GLIS3 acts as both a repressor and activator of gene transcription by binding to specific DNA sequences. It plays critical roles during embryonic development, particularly in the pancreas, thyroid, eye, liver, and kidney. Mutations in GLIS3 lead to neonatal diabetes and congenital hypothyroidism, with additional associations to other syndromic features such as polycystic kidney disease and facial abnormalities. The protein regulates cell specification and the expression of key hormones and transcription factors, notably controlling insulin gene expression in pancreatic beta cells. No approved drugs directly target GLIS3, but its genetic variants serve as important biomarkers for certain monogenic diseases.

Other names
ZNF515NDH
02

Biological functions

Transcriptional regulation (represses and activates gene expression)Pancreatic beta cell development and maturationThyroid developmentEye, liver, and kidney developmentRegulation of insulin gene expressionCell lineage specification
03

Disease associations

Neonatal diabetes mellitusCongenital hypothyroidismDiabetes mellitusPolycystic kidney diseaseThyroid abnormalitiesLiver fibrosisGlaucomaFacial abnormalities
04

Safety considerations

Potential risks in modulating GLIS3 include disruption of insulin production, thyroid function, kidney and liver development, and risk of inducing congenital syndromes
05

Biomarkers

Mutations or altered expression of GLIS3 in patient samples (used for diagnosis of neonatal diabetes and congenital hypothyroidism)

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