TY  - JOUR
A1  - Sekulovski, Samoil
A1  - Trowitzsch, Simon
T1  - Transfer RNA processing – from a structural and disease perspective
T2  - Biological chemistry
N2  - Transfer RNAs (tRNAs) are highly structured non-coding RNAs which play key roles in translation and cellular homeostasis. tRNAs are initially transcribed as precursor molecules and mature by tightly controlled, multistep processes that involve the removal of flanking and intervening sequences, over 100 base modifications, addition of non-templated nucleotides and aminoacylation. These molecular events are intertwined with the nucleocy- toplasmic shuttling of tRNAs to make them available at translating ribosomes. Defects in tRNA processing are linked to the development of neurodegenerative disorders. Here, we summarize structural aspects of tRNA processing steps with a special emphasis on intron-containing tRNA splicing involving tRNA splicing endonuclease and ligase. Their role in neurological pathologies will be discussed. Identification of novel RNA substrates of the tRNA splicing machinery has uncovered functions unrelated to tRNA processing. Future structural and biochemical studies will unravel their mechanistic underpinnings and deepen our understanding of neurological diseases.
KW  - end maturation
KW  - endonuclease
KW  - neurodegenerative disorders
KW  - RNA processing
KW  - structural biology
KW  - tRNA splicing
Y1  - 2022
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/62894
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-628949
SN  - 1437-4315
N1  - This work was financially supported by the German Research Foundation (DFG, grant number TR 1711/1-7), the Collaborative Research Center 902 (SFB902) ‘Molecular Principles of RNA-based Regulation’, and the Boehringer Ingelheim Fonds.
VL  - 403
IS  - 8–9
SP  - 749
EP  - 763
PB  - de Gruyter
CY  - Berlin [u.a.]
ER  -