REXO2

Chr 11q23.2

RNA exonuclease 2

Aliases:: DKFZP566E144, SFN, CGI-114
MANE:: ENST00000265881.10

OMIM: 607149 ↗NCBI: 25996 ↗Ensembl ↗

Annotations refreshed 10 hours ago.

Predicted protein structure

AlphaFold predicted structure

REXO2 · Q9Y3B8

v62025-08-01

Predicted Aligned Error matrix for REXO2 — diagonal shows residue index, colour shows pairwise prediction confidence (lower is better) — Predicted Aligned Error

Mean pLDDT

88.2/ 100

Confident

237 residues

Confidence breakdown

Very high(≥ 90)73%
Confident(70–90)10%
Low(50–70)11%
Very low(< 50)7%

Open interactive 3D viewer

AlphaFold (Jumper et al., 2021) · CC BY 4.0

Clinical relevance (Genomics England PanelApp)

Moderate Evidence (Amber)

Primary immunodeficiency or monogenic inflammatory bowel disease
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted

Disease associations (Open Targets)

neurodegenerative disease
0.51
lysosomal storage disease
0.26
type 1 interferonopathy
0.18
Hyperlipoproteinemia type 1
0.05
hyperlipoproteinemia type V
0.05
Hyperlipoproteinemia type 5
0.05
LCAT deficiency
0.05
glioma
0.05
central nervous system cancer
0.05
amegakaryocytic thrombocytopenia, congenital, 2
0.05

Score is the Open Targets composite evidence score (0-1). Higher = stronger gene-disease association.

Protein function (UniProt)

Oligoribonuclease, mitochondrial

3'-to-5'exoribonuclease that preferentially degrades DNA and RNA oligonucleotides composed of only two nucleotides (PubMed:23741365, PubMed:30926754, PubMed:31588022, PubMed:32365187). Binds and degrades longer oligonucleotides with a lower affinity (PubMed:30926754, PubMed:31588022, PubMed:32365187). 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 (PubMed:30926754, PubMed:31588022, PubMed:32365187). Essential for correct initiation of mitochondrial transcription, degrading mitochondrial RNA dinucleotides to prevent RNA-primed transcription at non-canonical sites in the mitochondrial genome (PubMed:31588022). Essential for embryonic development (By similarity)