CDK5RAP1
Function
Methylthiotransferase that catalyzes the conversion of N6-(dimethylallyl)adenosine (i(6)A) to 2-methylthio-N6-(dimethylallyl)adenosine (ms(2)i(6)A) 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).
Sequence Similarities
Belongs to the methylthiotransferase family. MiaB subfamily.
Tissue Specificity
Expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas (PubMed:10721722). Expressed in neurons of central nervous tissue (PubMed:10721722, PubMed:11882646).
Isoform 5
Mainly expressed in brain, placenta and testis.
Isoform 6
High expression in placenta and lung.
Cellular localization
- Mitochondrion
Alternative names
C20orf34, CGI-05, HSPC167, CDK5RAP1, Mitochondrial tRNA methylthiotransferase CDK5RAP1, CDK5 activator-binding protein C42, CDK5 regulatory subunit-associated protein 1, mt-tRNA-2-methylthio-N6-dimethylallyladenosine synthase, mt-tRNA-N6-(dimethylallyl)adenosine(37) methylthiotransferase