| 3236 | Otc | ornithine transcarbamylase | ENCODES a protein that exhibits amino acid binding; identical protein binding; ornithine carbamoyltransferase activity; INVOLVED IN citrulline biosynthetic process; liver development; midgut development; PARTICIPATES IN urea cycle pathway; AGAT deficiency pathway; arginine and proline metabolic path way; ASSOCIATED WITH Acidoses; alcohol-associated liver disease; Animal Hepatitis; FOUND IN mitochondrial inner membrane; mitochondrion (ortholog); INTERACTS WITH (+)-schisandrin B; 1-naphthyl isothiocyanate; 17alpha-ethynylestradiol | X | 15126358 | 15202473 | Rat | 255 | symbol , old_gene_name , PhenoGen | gene, protein-coding, PROVISIONAL [RefSeq] |
| 11039 | Otc | ornithine transcarbamylase | Predicted to enable several functions, including identical protein binding activity; ornithine carbamoyltransferase activity; and phosphate ion binding activity. Predicted to be involved in several processes, including ammonium homeostasis; non-proteinogenic amino acid metabolic process; and urea cy cle. Located in mitochondrial inner membrane. Is expressed in alimentary system; intestine; intrahepatic bile duct epithelium; liver; and small intestine. Used to study ornithine carbamoyltransferase deficiency. Human ortholog(s) of this gene implicated in ornithine carbamoyltransferase deficiency. Orthologous to human OTC (ornithine transcarbamylase). [provided by Alliance of Genome Resources, Apr 2025] | X | 10118584 | 10187275 | Mouse | 410 | symbol , old_gene_name , PhenoGen , description | gene, protein-coding, VALIDATED [RefSeq] |
| 732939 | OTC | ornithine transcarbamylase | This nuclear gene encodes a mitochondrial matrix enzyme. The encoded protein is involved in the urea cycle which functions to detoxify ammonia into urea for excretion. Mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia. [provided by RefSeq, May 2022] | X | 38327684 | 38422928 | Human | 511 | symbol , old_gene_name , COSMIC , Human Proteome Map , old_gene_symbol | gene, protein-coding, REVIEWED [RefSeq] |
| 8775465 | Otc | ornithine transcarbamylase | ENCODES a protein that exhibits amino acid binding (ortholog); identical protein binding (ortholog); ornithine carbamoyltransferase activity (ortholog); INVOLVED IN ammonium homeostasis (ortholog); citrulline biosynthetic process (ortholog); L-ornithine catabolic process (ortholog); PARTICIPATES IN AGAT deficiency pathway; arginine and proline metabolic pathway; argininosuccinic aciduria pathway; ASSOCIATED WITH Acidoses (ortholog); alcohol-associated liver disease (ortholog); Animal Hepatitis (ortholog); FOUND IN mitochondrial inner membrane (ortholog); mitochondrion (ortholog) | NW_004955601 | 481524 | 543176 | Chinchilla | 64 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 11868874 | OTC | ornithine transcarbamylase | ENCODES a protein that exhibits amino acid binding (ortholog); identical protein binding (ortholog); ornithine carbamoyltransferase activity (ortholog); INVOLVED IN citrulline biosynthetic process (ortholog); liver development (ortholog); midgut development (ortholog); PARTICIPATES IN AGAT deficienc y pathway; arginine and proline metabolic pathway; argininosuccinic aciduria pathway; ASSOCIATED WITH Acidoses (ortholog); alcohol-associated liver disease (ortholog); Animal Hepatitis (ortholog); FOUND IN mitochondrial inner membrane (ortholog) | X | 30796828 | 30868144 | Bonobo | 81 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12341549 | OTC | ornithine transcarbamylase | ENCODES a protein that exhibits amino acid binding (ortholog); identical protein binding (ortholog); ornithine carbamoyltransferase activity (ortholog); INVOLVED IN citrulline biosynthetic process (ortholog); liver development (ortholog); midgut development (ortholog); PARTICIPATES IN AGAT deficienc y pathway; arginine and proline metabolic pathway; argininosuccinic aciduria pathway; ASSOCIATED WITH Acidoses (ortholog); alcohol-associated liver disease (ortholog); Animal Hepatitis (ortholog); FOUND IN mitochondrial inner membrane (ortholog) | X | 33130921 | 33200441 | Dog | 82 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12562252 | Otc | ornithine transcarbamylase | ENCODES a protein that exhibits amino acid binding (ortholog); identical protein binding (ortholog); ornithine carbamoyltransferase activity (ortholog); INVOLVED IN citrulline biosynthetic process (ortholog); liver development (ortholog); midgut development (ortholog); PARTICIPATES IN AGAT deficienc y pathway; arginine and proline metabolic pathway; argininosuccinic aciduria pathway; ASSOCIATED WITH Acidoses (ortholog); alcohol-associated liver disease (ortholog); Animal Hepatitis (ortholog); FOUND IN mitochondrial inner membrane (ortholog) | NW_004936502 | 5234253 | 5291891 | Squirrel | 81 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 155237247 | otc | | | | | | Tropical Clawed Frog | | symbol | gene, null |
| 18925332 | Otc | ornithine transcarbamylase | ENCODES a protein that exhibits amino acid binding (ortholog); identical protein binding (ortholog); ornithine carbamoyltransferase activity (ortholog); INVOLVED IN citrulline biosynthetic process (ortholog); liver development (ortholog); midgut development (ortholog); PARTICIPATES IN AGAT deficienc y pathway; arginine and proline metabolic pathway; argininosuccinic aciduria pathway; ASSOCIATED WITH Acidoses (ortholog); alcohol-associated liver disease (ortholog); Animal Hepatitis (ortholog); FOUND IN mitochondrial inner membrane (ortholog) | | | | Naked Mole-Rat | 81 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 625999852 | Otc | ornithine transcarbamylase | ENCODES a protein that exhibits amino acid binding (ortholog); identical protein binding (ortholog); ornithine carbamoyltransferase activity (ortholog); INVOLVED IN ammonium homeostasis (ortholog); citrulline biosynthetic process (ortholog); L-ornithine catabolic process (ortholog); PARTICIPATES IN AGAT deficiency pathway; arginine and proline metabolic pathway; argininosuccinic aciduria pathway; ASSOCIATED WITH Acidoses (ortholog); alcohol-associated liver disease (ortholog); Animal Hepatitis (ortholog); FOUND IN mitochondrial inner membrane (ortholog); mitochondrion (ortholog) | | | | Black Rat | 64 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 14195640 | OTC | ornithine transcarbamylase | ENCODES a protein that exhibits amino acid binding (ortholog); identical protein binding (ortholog); ornithine carbamoyltransferase activity (ortholog); INVOLVED IN citrulline biosynthetic process (ortholog); liver development (ortholog); midgut development (ortholog); PARTICIPATES IN AGAT deficienc y pathway; arginine and proline metabolic pathway; argininosuccinic aciduria pathway; ASSOCIATED WITH Acidoses (ortholog); alcohol-associated liver disease (ortholog); Animal Hepatitis (ortholog); FOUND IN mitochondrial inner membrane (ortholog); mitochondrion (ortholog) | | | | Pig | 86 | symbol , old_gene_name | gene, protein-coding, PROVISIONAL [RefSeq] |
| 18533324 | OTC | ornithine transcarbamylase | ENCODES a protein that exhibits amino acid binding (ortholog); identical protein binding (ortholog); ornithine carbamoyltransferase activity (ortholog); INVOLVED IN citrulline biosynthetic process (ortholog); liver development (ortholog); midgut development (ortholog); PARTICIPATES IN AGAT deficienc y pathway; arginine and proline metabolic pathway; argininosuccinic aciduria pathway; ASSOCIATED WITH Acidoses (ortholog); alcohol-associated liver disease (ortholog); Animal Hepatitis (ortholog); FOUND IN mitochondrial inner membrane (ortholog) | | | | Green Monkey | 81 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 155257087 | otc.S | | | | | | African Clawed Frog | | symbol | gene, null |