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Structure paper

TitleStructural basis of 3'-tRNA maturation by the human mitochondrial RNase Z complex.
Journal, issue, pagesEMBO J, Vol. 43, Issue 24, Page 6573-6590, Year 2024
Publish dateNov 8, 2024
AuthorsGenís Valentín Gesé / B Martin Hällberg /
PubMed AbstractMaturation of human mitochondrial tRNA is essential for cellular energy production, yet the underlying mechanisms remain only partially understood. Here, we present several cryo-EM structures of the ...Maturation of human mitochondrial tRNA is essential for cellular energy production, yet the underlying mechanisms remain only partially understood. Here, we present several cryo-EM structures of the mitochondrial RNase Z complex (ELAC2/SDR5C1/TRMT10C) bound to different maturation states of mitochondrial tRNA, showing the molecular basis for tRNA-substrate selection and catalysis. Our structural insights provide a molecular rationale for the 5'-to-3' tRNA processing order in mitochondria, the 3'-CCA antideterminant effect, and the basis for sequence-independent recognition of mitochondrial tRNA substrates. Furthermore, our study links mutations in ELAC2 to clinically relevant mitochondrial diseases, offering a deeper understanding of the molecular defects contributing to these conditions.
External linksEMBO J / PubMed:39516281 / PubMed Central
MethodsEM (single particle)
Resolution1.9 - 3.34 Å
Structure data

EMDB-19954: Human mitochondrial RNase Z with tRNA-His, ELAC2 focused map
Method: EM (single particle) / Resolution: 3.07 Å

EMDB-19955: Human mitochondrial RNase Z with tRNA-His, consensus map
Method: EM (single particle) / Resolution: 2.78 Å

EMDB-19956: Human mitochondrial RNase Z with tRNA-His precursor, consensus map
Method: EM (single particle) / Resolution: 2.69 Å

EMDB-19957: Human mitochondrial RNase Z with tRNA-His precursor, ELAC2 focused map
Method: EM (single particle) / Resolution: 3.34 Å

EMDB-19958: Human mitochondrial RNase Z with tRNA-His-CCA, consensus map
Method: EM (single particle) / Resolution: 3.08 Å

EMDB-19959: Human mitochondrial RNase Z with tRNA-His-CCA, ELAC2 focused map
Method: EM (single particle) / Resolution: 3.2 Å

50050

9ey0

EMDB-50050, PDB-9ey0:
Human mitochondrial RNase Z with tRNA-His
Method: EM (single particle) / Resolution: 2.78 Å

50051

9ey1

EMDB-50051, PDB-9ey1:
Human mitochondrial RNase Z with tRNA-His precursor
Method: EM (single particle) / Resolution: 2.9 Å

50052

9ey2

EMDB-50052, PDB-9ey2:
Human mitochondrial RNase Z with tRNA-His-CCA
Method: EM (single particle) / Resolution: 2.96 Å

51230

9gch

EMDB-51230, PDB-9gch:
Human mitochondrial RNase Z with tRNA-His-CCA, SDR5C1/TRMT10C focus
Method: EM (single particle) / Resolution: 1.9 Å

Chemicals

ChemComp-ZN:
Unknown entry

ChemComp-GTP:
GUANOSINE-5'-TRIPHOSPHATE / GTP, energy-carrying molecule*YM

ChemComp-SAM:
S-ADENOSYLMETHIONINE

ChemComp-SAH:
S-ADENOSYL-L-HOMOCYSTEINE

ChemComp-MG:
Unknown entry

ChemComp-HOH:
WATER

Source
  • homo sapiens (human)
KeywordsRNA BINDING PROTEIN / RNAseZ / ELAC2 / mitochondria / tRNA / RNase Z

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