CDK5RAP1

CDK5RAP1 (CDK5 Regulatory Subunit Associated Protein 1) is a Protein Coding gene. Gene Ontology (GO) annotations related to this gene include protein kinase binding and transferase activity. An important paralog of this gene is CDKAL1.

Full Name

CDK5 Regulatory Subunit Associated Protein 1

Function

Methylthiotransferase that catalyzes the conversion of N6-(dimethylallyl)adenosine (i6A) to 2-methylthio-N6-(dimethylallyl)adenosine (ms2i6A) at position 37 (adjacent to the 3'-end of the anticodon) of four mitochondrial DNA-encoded tRNAs (Ser(UCN), Phe, Tyr and Trp) (PubMed:22422838, PubMed:25738458, PubMed:28981754).
Essential for efficient and highly accurate protein translation by the ribosome (PubMed:22422838, PubMed:25738458, PubMed:28981754).
Specifically inhibits CDK5 activation by CDK5R1 (PubMed:11882646).
Essential for efficient mitochondrial protein synthesis and respiratory chain; shows pathological consequences in mitochondrial disease (PubMed:25738458).

Biological Process

Brain development Source: UniProtKB
Mitochondrial tRNA modification Source: Ensembl
Negative regulation of cyclin-dependent protein serine/threonine kinase activity Source: UniProtKB
Positive regulation of cyclin-dependent protein serine/threonine kinase activity Source: Ensembl
Positive regulation of mitochondrial translation Source: Ensembl
Positive regulation of translational fidelity Source: Ensembl
Regulation of neuron differentiation Source: UniProtKB

Cellular Location

Mitochondrion