GLIS2
Description
The GLIS2 (GLIS family zinc finger 2) is a protein-coding gene located on chromosome 16.
GLIS2, also known as GLIS family zinc finger 2, is a human gene encoding a Kruppel-like transcription factor. It acts as an activator or repressor of gene transcription depending on the gene and promoter context. GLIS2 plays a role in kidney development and neurogenesis. Mice lacking GLIS2 exhibit reduced size and weight, with their kidneys displaying progressive atrophy and symptoms similar to human nephronophthisis. GLIS2 is essential for maintaining renal tissue integrity by preventing apoptosis and fibrosis. Mutations in the GLIS2 gene are associated with nephronophthisis.
GLIS2 acts as a transcriptional regulator, functioning as either a repressor or an activator depending on the cellular context. It plays a role in inhibiting the Hedgehog signaling pathway by suppressing the expression of Wnt4, a downstream target of Hedgehog signaling. Additionally, GLIS2 is crucial for maintaining the differentiated epithelial phenotype in renal cells by inhibiting SNAI1, a protein that promotes epithelial-to-mesenchymal transition. Furthermore, GLIS2 represses transcriptional activation mediated by CTNNB1 in the Wnt signaling pathway, potentially by recruiting the corepressors CTBP1 and HDAC3. GLIS2 may also be involved in neuron differentiation.
GLIS2 is also known as NKL, NPHP7.
