Journal
MITOCHONDRION
Volume 9, Issue 4, Pages 284-288Publisher
ELSEVIER SCI LTD
DOI: 10.1016/j.mito.2009.03.008
Keywords
RNase P; tRNA; mtDNA disease; Alzheimer's disease
Categories
Funding
- Austrian Science Fund (FWF) [P17453]
- Austrian Science Fund (FWF) [W1207] Funding Source: Austrian Science Fund (FWF)
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RNase P is the endonuclease responsible for the maturation of the 5' ends of tRNAs. A catalytic RNA component was long considered the premier attribute of the enzyme family. Ignoring this heritage, human mitochondria make their RNase P of three proteins only. While one of them appears to be the metallonuclease actually responsible for phosphodiester hydrolysis, the other two have been recruited from unrelated biochemical pathways and may be critical for substrate recognition. One of them is moreover identical to a previously identified amyloid-beta-binding protein, whereby it could link tRNA processing to mitochondrial dysfunction in Alzheimer's disease. (C) 2009 Elsevier B.V. and Mitochondria Research Society. All rights reserved.
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