POLR3A Gene

POLR3A (RNA polymerase III subunit A) is a protein-coding gene located on chromosome . 10q22.3. The protein encoded by the POLR3A gene is the catalytic component of RNA polymerase III, which synthesizes small RNAs. The encoded protein also functions as a sensor to recognize foreign DNA and elicit an innate immune response.

Diseases associated with POLR3A include.

• Leukodystrophy, hypomyelinating, 7, with or without oligodontia
• and the
• Wiedemann-Rautenstrauch syndrome (OMIM: 264090/, a rare autosomal recessive neonatal progeroid disorder characterized by intrauterine growth retardation, failure to thrive, short stature, progeroid appearance, hypotonia, and variable mental impairment.

DNA-dependent RNA polymerase catalyzes the transcription of DNA into RNA using the four ribonucleoside triphosphates as substrates. Together with the second largest subunit, forms the active site of the polymerase. Plays a key role in the recognition and limitation of infection by intracellular bacteria and DNA viruses. Acts as a nuclear and cytosolic DNA sensor involved in the innate immune response. Can recognize non-self dsDNA, which serves as a template for transcription into dsRNA.