A Review of PRDM13 Transcriptional Regulation in Vertebrate Retinal Development and Disease

The retina is a complex neuronal tissue whose development from a common retinal progenitor cell population is tightly controlled by a complex gene regulatory network driven by transcription factors. PRDM13 is a transcription factor known to play a critical role in the specification of neurons across the central nervous system, and its expression is maintained into adulthood in a limited number of neuronal types, including neurons of the dorsomedial hypothalamus and retinal amacrine cells. Dysregulation of PRDM13 has been associated with three inherited human maculopathies - North Carolina macular dystrophy (NCMD), progressive bifocal chorioretinal atrophy (PBCRA), and congenital posterior polar chorioretinal hypoplasia (CPPCRH) - indicating that PRDM13 may also play a role in development and maintenance of non-amacrine retinal cell types. Herein, we review the known roles of PRDM13 across the developing central nervous system to provide a comprehensive understanding of PRDM13's general and tissue-specific functions, as well as how PRDM13 dysregulation may lead to improper macular development and disease. Future studies into PRDM13 and its downstream regulatory network will be important to provide critical insights into the regulation of cell fate decisions in the developing retina.