POLR3G

RNA polymerase III subunit G (POLR3G, alias RPC7α) is a core component of the RNA polymerase III (Pol III) enzyme complex. Pol III mediates transcription of small non-coding RNAs including transfer RNAs (tRNAs), 5S ribosomal RNA, and U6 small nuclear RNA—molecules critical for protein translation and pre-mRNA splicing. POLR3G supports the structural stability and catalytic activity of the Pol III complex, ensuring efficient synthesis of these essential transcripts.
Pathogenic mutations in POLR3G are linked to POLR3-related hypomyelinating leukodystrophy (POLR3-HLD), a rare neurodegenerative disorder marked by impaired central nervous system myelination. While no targeted therapies for POLR3G-associated conditions are approved, preclinical research explores Pol III subunit inhibition for cancers (where Pol III activity is upregulated) and gene-editing strategies to correct leukodystrophy-causing mutations.