Publication: Gli-similar (Glis) Krüppel-like zinc finger proteins:
insights into their physiological functions and critical
roles in neonatal diabetes and cystic renal disease
Authors
Hong Soon, Kang ; ZeRuth, Gary ; Lichti-Kaiser, Kristin ; Vasanth, Shivakumar ; Yin, Zhengyu ; Kim, Yong-Sik ; Jetten, Anton M.
item.page.secondaryauthor
item.page.director
Publisher
Murcia : F. Hernández
publication.page.editor
publication.page.department
DOI
item.page.type
info:eu-repo/semantics/article
Description
Abstract
GLI-similar (Glis) 1-3 proteins constitute a
subfamily of the Krüppel-like zinc finger transcription
factors that are closely related to the Gli family. Glis1-3
play critical roles in the regulation of a number of
physiological processes and have been implicated in
several pathologies. Mutations in GLIS2 have been
linked to nephronophthisis, an autosomal recessive
cystic kidney disease. Loss of Glis2 function leads to
renal atrophy and fibrosis that involves epithelialmesenchymal
transition (EMT) of renal tubule epithelial
cells. Mutations in human GLIS3 have been implicated
in a syndrome characterized by neonatal diabetes and
congenital hypothyroidism (NDH) and in some patients
accompanied by polycystic kidney disease, glaucoma,
and liver fibrosis. In addition, the GLIS3 gene has been
identified as a susceptibility locus for the risk of type 1
and 2 diabetes. Glis3 plays a key role in pancreatic
development, particularly in the generation of ß-cells
and in the regulation of insulin gene expression. Glis2
and Glis3 proteins have been demonstrated to localize to
the primary cilium, a signaling organelle that has been
implicated in several pathologies, including cystic renal
diseases. This association suggests that Glis2/3 are part
of primary cilium-associated signaling pathways that
control the activity of Glis proteins. Upon activation in
the primary cilium, Glis proteins may translocate to the
nucleus where they subsequently regulate gene
transcription by interacting with Glis-binding sites in the
promoter regulatory region of target genes. In this
review, we discuss the current knowledge of the Glis
signaling pathways, their physiological functions, and
their involvement in several human pathologies.
publication.page.subject
Citation
item.page.embargo
Ir a Estadísticas
Sin licencia Creative Commons.