| Modification Notes |
Zinc-finger proteins contain DNA-binding domains and have a wide variety of functions, most of which encompass some form of transcriptional activation or repression. GLIS3 (gLIS family zinc finger 3), also known as ZNF515 (zinc finger protein 515), is a 775 amino acid protein that localizes to the nucleus and contains five C2H2-type zinc fingers. Expressed in a variety of tissues, including kidney, brain, liver, lung, ovary, pancreas, thymus and skeletal muscle, GLIS3 functions as both an activator and a suppressor of transcription, specifically binding the consensus sequence 5'-GACCACCCAC-3' through its C2H2-type zinc fingers. Defects in the gene encoding GLIS3 are a cause of NDH syndrome; a neonatal diabetes that is characterized by congenital hypothyroidism, congenital glaucoma, hepatic fibrosis and polycystic kidneys. |