Protein: Mitochondrial tRNA methylthiotransferase CDK5RAP1 (Q96SZ6)
Organism: Homo sapiens (Human)
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
Subcellular location: Mitochondrion
Sequence: MHPLQCVLQVQRSLGWGPLASVSWLSLRMCRAHSSLSSTMCPSPERQEDGARKDFSSRLAAGPTFQHFLKSASAPQEKLSSEVEDPPPYLMMDELLGRQRKVYLETYGCQMNVNDTEIAWSILQKSGYLRTSNLQEADVILLVTCSIREKAEQTIWNRLHQLKALKTRRPRSRVPLRIGILGCMAERLKEEILNREKMVDILAGPDAYRDLPRLLAVAESGQQAANVLLSLDETYADVMPVQTSASATSAFVSIMRGCDNMCSYCIVPFTRGRERSRPIASILEEVKKLSEQVFLPPRPPKVLGLQGLKEVTLLGQNVNSFRDNSEVQFNSAVPTNLSRGFTTNYKTKQGGLRFAHLLDQVSRVDPEMRIRFTSPHPKDFPDEVLQLIHERDNICKQIHLPAQSGSSRVLEAMRRGYSREAYVELVHHIRESIPGVSLSSDFIAGFCGETEEDHVQTVSLLREVQYNMGFLFAYSMRQKTRAYHRLKDDVPEEVKLRRLEELITIFREEATKANQTSVGCTQLVLVEGLSKRSATDLCGRNDGNLKVIFPDAEMEDVNNPGLRVRAQPGDYVLVKITSASSQTLRGHVLCRTTLRDSSAYC
InterPro domains: - IPR006463: tRNA-2-methylthio-N(6)-dimethylallyladenosine synthase MiaB (family) [98-591]
- IPR038135: Methylthiotransferase, N-terminal domain superfamily (homologous_superfamily) [100-234]
- IPR005839: Methylthiotransferase (family) [98-594]
- IPR058240: Radical SAM superfamily (homologous_superfamily) [245-508]
- IPR020612: Methylthiotransferase, conserved site (conserved_site) [252-272]
- IPR002792: TRAM domain (domain) [515-590]
- IPR006638: Elp3/MiaA/NifB-like, radical SAM core domain (domain) [248-501]
- IPR013848: Methylthiotransferase, N-terminal (domain) [100-220]
- IPR007197: Radical SAM (domain) [98-594]
- IPR023404: Radical SAM, alpha/beta horseshoe (homologous_superfamily) [246-518]
Interaction partners: - Exosome complex component RRP40
- Exosome complex component RRP46
- Cyclin-dependent kinase 5
- tRNA dimethylallyltransferase
- Cyclin-dependent kinase 5 activator 1
- Pre-mRNA-processing factor 40 homolog B
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0003824 (catalytic activity), GO:0016740 (transferase activity), GO:0051540 (metal cluster binding), GO:0016782 (transferase activity, transferring sulphur-containing groups), GO:0051536 (iron-sulfur cluster binding), GO:0051539 (4 iron, 4 sulfur cluster binding)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0009987 (cellular process), GO:0044237 (cellular metabolic process), GO:0071704 (organic substance metabolic process), GO:0044238 (primary metabolic process), GO:0006807 (nitrogen compound metabolic process), GO:0006725 (cellular aromatic compound metabolic process), GO:1901360 (organic cyclic compound metabolic process), GO:0034641 (cellular nitrogen compound metabolic process), GO:0006139 (nucleobase-containing compound metabolic process), GO:0043170 (macromolecule metabolic process), GO:0046483 (heterocycle metabolic process), GO:0043412 (macromolecule modification), GO:0090304 (nucleic acid metabolic process), GO:0010467 (gene expression), GO:0016070 (RNA metabolic process), GO:0006396 (RNA processing), GO:0009451 (RNA modification), GO:0006400 (tRNA modification), GO:0034470 (ncRNA processing), GO:0034660 (ncRNA metabolic process), GO:0006399 (tRNA metabolic process), GO:0008033 (tRNA processing)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0005737 (cytoplasm), GO:0031974 (membrane-enclosed lumen), GO:0005739 (mitochondrion), GO:0043233 (organelle lumen), GO:0043229 (intracellular organelle), GO:0043227 (membrane-bounded organelle), GO:0043231 (intracellular membrane-bounded organelle), GO:0005759 (mitochondrial matrix), GO:0070013 (intracellular organelle lumen)