GO_REF:0000002
Gene Ontology annotation through association of InterPro records with GO terms
GO_REF:0000033
Annotation inferences using phylogenetic trees
GO_REF:0000044
Gene Ontology annotation based on UniProtKB/Swiss-Prot Subcellular Location vocabulary mapping, accompanied by conservative changes to GO terms applied by UniProt
GO_REF:0000052
Gene Ontology annotation based on curation of immunofluorescence data
GO_REF:0000107
Automatic transfer of experimentally verified manual GO annotation data to orthologs using Ensembl Compara
GO_REF:0000117
Electronic Gene Ontology annotations created by ARBA machine learning models
GO_REF:0000120
Combined Automated Annotation using Multiple IEA Methods
PMID:2556444
An arginine to glutamine mutation in residue 109 of human ornithine transcarbamylase completely abolishes enzymatic activity in Cos1 cells.
PMID:2575934
Structure of the ornithine transcarbamylase (OTC) gene and DNA diagnosis of OTC deficiency.
PMID:3472484
Targeting of nuclear-encoded proteins to the mitochondrial matrix: implications for human genetic defects.
PMID:34800366
Quantitative high-confidence human mitochondrial proteome and its dynamics in cellular context.
PMID:3895227
Arginine in the leader peptide is required for both import and proteolytic cleavage of a mitochondrial precursor.
PMID:6372096
Structure and expression of a complementary DNA for the nuclear coded precursor of human mitochondrial ornithine transcarbamylase.
PMID:8112735
Expression of four mutant human ornithine transcarbamylase genes in cultured Cos 1 cells relates to clinical phenotypes.
Reactome:R-HSA-70560
carbamoyl phosphate + ornithine => citrulline + orthophosphate
Reactome:R-HSA-8986181
PITRM1 proteolyzes mitochondrial targeting peptides (presequences)
Reactome:R-HSA-9956502
OTC precursor is targeted to the mitochondrial matrix and processed
Reactome:R-HSA-9956513
Leader sequence variants of OTC aren't targeted to the mitochondria
Reactome:R-HSA-9956527
OTC variants don't synthesize L-citrulline
Reactome:R-HSA-9959881
OTC gene expression