REXO2
Description
The REXO2 (RNA exonuclease 2) is a protein-coding gene located on chromosome 11.
REX02, RNA exonuclease 2 homolog (S. cerevisiae), also known as REXO2, is an enzyme which in humans is encoded by the REXO2 gene. Nucleases are components of DNA and RNA metabolism that carry out functions in DNA repair, replication, and recombination and in RNA processing and degradation. REX02 is a homolog of Orn, a 3-prime-to-5-prime exoribonuclease of E. coli that attacks the free 3-prime hydroxyl group on single-stranded RNA, releasing 5-prime mono-nucleotides in a sequential manner.
REX02 is a 3'-to-5' exoribonuclease that preferentially degrades DNA and RNA oligonucleotides composed of only two nucleotides. It binds and degrades longer oligonucleotides with a lower affinity. REX02 plays dual roles in mitochondria, scavenging nanoRNAs (small RNA oligonucleotides of <5 nucleotides) that are produced by the degradosome and clearing short RNAs that are generated by RNA processing. It is essential for correct initiation of mitochondrial transcription, degrading mitochondrial RNA dinucleotides to prevent RNA-primed transcription at non-canonical sites in the mitochondrial genome. It is essential for embryonic development.
REXO2 is also known as CGI-114, REX2, RFN, SFN.
Associated Diseases
- lysosomal storage disease
- cholesterol-ester transfer protein deficiency
- hypertriglyceridemia 2
- glycogen storage disease VI
- hyperinsulinism due to INSR deficiency
- familial apolipoprotein C-II deficiency
- sitosterolemia
- megaloblastic anemia, folate-responsive
- thyroid hormone metabolism, abnormal, 2
- homozygous familial hypercholesterolemia
- glycogen storage disease IXa1
- coronary artery disease, autosomal dominant 2
- disabling pansclerotic morphea of childhood
- congenital diarrhea 7 with exudative enteropathy
