Recombinant Human CDK5 regulatory subunit-associated protein 1 (CDK5RAP1)

Uniprot Id

Q96SZ6

Target Species

Human

Target Name

CDK5RAP1

Target Full Name

Mitochondrial tRNA methylthiotransferase CDK5RAP1

Target 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). Essential for efficient and highly accurate protein translation by the ribosome. Specifically inhibits CDK5 activation by CDK5R1. Essential for efficient mitochondrial protein synthesis and respiratory chain; shows pathological consequences in mitochondrial disease.

Target Subcellular Location

Mitochondrion.

Target Protein Families

Methylthiotransferase family, MiaB subfamily

Target Tissue Specificity

Expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas. Expressed in neurons of central nervous tissue.; [Isoform 5]: Mainly expressed in brain, placenta and testis.; [Isoform 6]: High expression in placenta and lung.

Target Synonyms

CDK5RAP1; C20orf34; CGI-05; HSPC167CDK5 regulatory subunit-associated protein 1; CDK5 activator-binding protein C42

Target Background

This gene encodes a regulator of cyclin-dependent kinase 5 activity. This protein has also been reported to modify RNA by adding a methylthio-group and may thus have a dual function as an RNA methylthiotransferase and as an inhibitor of cyclin-dependent kinase 5 activity. Alternative splicing results in multiple transcript variants that encode different isoforms.