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Metabolomics,Unknown,Transcriptomics,Genomics,Proteomics

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TRNA fragment profiling in CLP1 mutant (kinase-dead) mice

ABSTRACT: Loss of CLP1 activity results in the accumulation of novel sets of small RNA fragments derived from aberrant processing of tyrosine pre-tRNA. Such tRNA fragments sensitize cells to oxidative stress-induced p53 activation and p53-dependent cell death. 2 samples, Wt and Clp1(k/k), no replicates

ORGANISM(S): Mus musculus

SUBMITTER: Javier Martinez

PROVIDER: E-GEOD-39275 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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CLP1 links tRNA metabolism to progressive motor-neuron loss.

Hanada Toshikatsu T Weitzer Stefan S Mair Barbara B Bernreuther Christian C Wainger Brian J BJ Ichida Justin J Hanada Reiko R Orthofer Michael M Cronin Shane J SJ Komnenovic Vukoslav V Minis Adi A Sato Fuminori F Mimata Hiromitsu H Yoshimura Akihiko A Tamir Ido I Rainer Johannes J Kofler Reinhard R Yaron Avraham A Eggan Kevin C KC Woolf Clifford J CJ Glatzel Markus M Herbst Ruth R Martinez Javier J Penninger Josef M JM

Nature 20130310 7442

CLP1 was the first mammalian RNA kinase to be identified. However, determining its in vivo function has been elusive. Here we generated kinase-dead Clp1 (Clp1(K/K)) mice that show a progressive loss of spinal motor neurons associated with axonal degeneration in the peripheral nerves and denervation of neuromuscular junctions, resulting in impaired motor function, muscle weakness, paralysis and fatal respiratory failure. Transgenic rescue experiments show that CLP1 functions in motor neurons. Mec ...[more]

PMID: 23474986

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