| 628882 | Elac2 | elaC ribonuclease Z 2 | ENCODES a protein that exhibits tRNA-specific ribonuclease activity (ortholog); INVOLVED IN mitochondrial tRNA 3'-end processing (ortholog); tRNA 3'-end processing (ortholog); PARTICIPATES IN tRNA maturation pathway; RNA transport pathway; ASSOCIATED WITH combined oxidative phosphorylation deficienc y 17 (ortholog); congenital disorder of glycosylation type IIn (ortholog); corticosterone methyloxidase deficiency 1 (ortholog); FOUND IN mitochondrial matrix (ortholog); mitochondrial nucleoid (ortholog); mitochondrion (ortholog); INTERACTS WITH 2,3,7,8-tetrachlorodibenzodioxine; aconitine; allethrin | 10 | 50131449 | 50154755 | Rat | 134 | symbol , PhenoGen | gene, protein-coding, VALIDATED [RefSeq] |
| 1342597 | ELAC2 | elaC ribonuclease Z 2 | 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 k nown 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] | 17 | 12991612 | 13018027 | Human | 210 | symbol , old_gene_name , COSMIC , Human Proteome Map | gene, protein-coding, REVIEWED [RefSeq] |
| 1552652 | Elac2 | elaC ribonuclease Z 2 | Predicted to enable 3'-tRNA processing endoribonuclease activity. Predicted to be involved in mitochondrial tRNA 3'-end processing. Located in mitochondrial nucleoid. Is expressed in extraembryonic component and placenta. Human ortholog(s) of this gene implicated in combined oxidative phosphorylatio n deficiency 17. Orthologous to human ELAC2 (elaC ribonuclease Z 2). [provided by Alliance of Genome Resources, Jul 2025] | 11 | 64869856 | 64892906 | Mouse | 151 | symbol , PhenoGen , description | gene, protein-coding, VALIDATED [RefSeq] |
| 8822883 | Elac2 | elaC ribonuclease Z 2 | ENCODES a protein that exhibits tRNA-specific ribonuclease activity (ortholog); INVOLVED IN mitochondrial tRNA 3'-end processing (ortholog); tRNA 3'-end processing (ortholog); PARTICIPATES IN tRNA maturation pathway; ASSOCIATED WITH combined oxidative phosphorylation deficiency 17 (ortholog); congen ital disorder of glycosylation type IIn (ortholog); corticosterone methyloxidase deficiency 1 (ortholog); FOUND IN endoribonuclease complex (ortholog); mitochondrial matrix (ortholog); mitochondrial nucleoid (ortholog) | NW_004955467 | 4406947 | 4428769 | Chinchilla | 28 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 11959846 | ELAC2 | elaC ribonuclease Z 2 | ENCODES a protein that exhibits tRNA-specific ribonuclease activity (ortholog); INVOLVED IN mitochondrial tRNA 3'-end processing (inferred); tRNA 3'-end processing (inferred); tRNA processing (inferred); PARTICIPATES IN tRNA maturation pathway; ASSOCIATED WITH combined oxidative phosphorylation defi ciency 17 (ortholog); congenital disorder of glycosylation type IIn (ortholog); corticosterone methyloxidase deficiency 1 (ortholog); FOUND IN mitochondrial matrix (inferred); mitochondrial nucleoid (inferred); mitochondrion (inferred) | 17 | 38631454 | 38657855 | Bonobo | 39 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12267073 | ELAC2 | elaC ribonuclease Z 2 | ENCODES a protein that exhibits tRNA-specific ribonuclease activity (ortholog); INVOLVED IN mitochondrial tRNA 3'-end processing (inferred); tRNA 3'-end processing (inferred); tRNA processing (inferred); PARTICIPATES IN tRNA maturation pathway; ASSOCIATED WITH combined oxidative phosphorylation defi ciency 17 (ortholog); congenital disorder of glycosylation type IIn (ortholog); corticosterone methyloxidase deficiency 1 (ortholog); FOUND IN mitochondrial matrix (inferred); mitochondrial nucleoid (inferred); mitochondrion (inferred) | 5 | 36957901 | 36978646 | Dog | 40 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12551222 | Elac2 | elaC ribonuclease Z 2 | ENCODES a protein that exhibits tRNA-specific ribonuclease activity (ortholog); INVOLVED IN mitochondrial tRNA 3'-end processing (inferred); tRNA 3'-end processing (inferred); tRNA processing (inferred); PARTICIPATES IN tRNA maturation pathway; ASSOCIATED WITH combined oxidative phosphorylation defi ciency 17 (ortholog); congenital disorder of glycosylation type IIn (ortholog); corticosterone methyloxidase deficiency 1 (ortholog); FOUND IN mitochondrial matrix (inferred); mitochondrial nucleoid (inferred); mitochondrion (inferred) | NW_004936595 | 5543476 | 5565075 | Squirrel | 39 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 155259637 | elac2 | | | | | | Tropical Clawed Frog | | symbol | gene, null |
| 14237010 | ELAC2 | elaC ribonuclease Z 2 | ENCODES a protein that exhibits tRNA-specific ribonuclease activity (ortholog); INVOLVED IN mitochondrial tRNA 3'-end processing (inferred); tRNA 3'-end processing (inferred); tRNA processing (inferred); PARTICIPATES IN tRNA maturation pathway; ASSOCIATED WITH combined oxidative phosphorylation defi ciency 17 (ortholog); congenital disorder of glycosylation type IIn (ortholog); corticosterone methyloxidase deficiency 1 (ortholog); FOUND IN mitochondrial matrix (inferred); mitochondrial nucleoid (inferred); mitochondrion (inferred) | | | | Pig | 40 | symbol | gene, protein-coding, VALIDATED [RefSeq] |
| 18360511 | ELAC2 | elaC ribonuclease Z 2 | ENCODES a protein that exhibits tRNA-specific ribonuclease activity (ortholog); INVOLVED IN mitochondrial tRNA 3'-end processing (inferred); tRNA 3'-end processing (inferred); tRNA processing (inferred); PARTICIPATES IN tRNA maturation pathway; ASSOCIATED WITH combined oxidative phosphorylation defi ciency 17 (ortholog); congenital disorder of glycosylation type IIn (ortholog); corticosterone methyloxidase deficiency 1 (ortholog); FOUND IN mitochondrial matrix (inferred); mitochondrial nucleoid (inferred); mitochondrion (inferred) | | | | Green Monkey | 39 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 18916864 | Elac2 | elaC ribonuclease Z 2 | ENCODES a protein that exhibits tRNA-specific ribonuclease activity (ortholog); INVOLVED IN tRNA 3'-end processing (ortholog); PARTICIPATES IN tRNA maturation pathway; ASSOCIATED WITH combined oxidative phosphorylation deficiency 17 (ortholog); congenital disorder of glycosylation type IIn (ortholog ); corticosterone methyloxidase deficiency 1 (ortholog); FOUND IN endoribonuclease complex (ortholog); mitochondrial matrix (ortholog); nucleoplasm (ortholog) | | | | Naked Mole-Rat | 36 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 626183979 | elac2.L | | | | | | African Clawed Frog | | symbol | gene, null |
| 626188732 | elac2.L | | | | | | African Clawed Frog | | symbol | gene, null |
| 628359259 | elac2.S | | | | | | African Clawed Frog | | symbol | gene, null |