Mitochondrial ribonuclease P catalytic subunit

Definition:

Catalytic ribonuclease component of mitochondrial ribonuclease P, a complex composed of TRMT10C/MRPP1, HSD17B10/MRPP2 and PRORP/MRPP3, which cleaves tRNA molecules in their 5'-ends. The presence of TRMT10C/MRPP1, HSD17B10/MRPP2 is required to catalyze tRNA molecules in their 5'-ends.

References:

[1]. Linda Reinhard, et al. Structure of the nuclease subunit of human mitochondrial RNase P. Nucleic Acids Res. 2015 Jun 23;43(11):5664-72. [Content Brief]

[2]. Johann Holzmann, et al. RNase P without RNA: identification and functional reconstitution of the human mitochondrial tRNA processing enzyme. Cell. 2008 Oct 31;135(3):462-74. [Content Brief]

[3]. Irit Hochberg, et al. Bi-allelic variants in the mitochondrial RNase P subunit PRORP cause mitochondrial tRNA processing defects and pleiotropic multisystem presentations. Am J Hum Genet. 2021 Nov 4;108(11):2195-2204. [Content Brief]

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