ELAC2

Chr 17ADAR

elaC ribonuclease Z 2

Also known as: COXPD17, ELC2, HPC2

The protein encoded by this gene has a C-terminal domain with tRNA 3′ processing endoribonuclease activity, which catalyzes the removal of the 3' trailer from precursor tRNAs. The protein also interacts with activated Smad family member 2 (Smad2) and its nuclear partner forkhead box H1 (also known as FAST-1), and reduced expression can suppress transforming growth factor-beta induced growth arrest. Mutations in this gene result in an increased risk of prostate cancer. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Sep 2009]

OMIMResearchGenerating clinical summary…
LOFmechanismAD/ARLOEUF 1.052 OMIM phenotypes
Clinical SummaryELAC2
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Gene-Disease Validity (ClinGen)
mitochondrial disease · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.05LOEUF
pLI 0.000
Z-score 1.30
OE 0.79 (0.611.05)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.41Z-score
OE missense 1.05 (0.981.14)
479 obs / 454.4 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.79 (0.611.05)
00.351.4
Missense OE?1.05 (0.981.14)
00.61.4
Synonymous OE?1.21
01.21.6
LoF obs/exp: 36 / 45.5Missense obs/exp: 479 / 454.4Syn Z: -2.25

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ELAC2 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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