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Fig. 3 | Orphanet Journal of Rare Diseases

Fig. 3

From: What’s new in pontocerebellar hypoplasia? An update on genes and subtypes

Fig. 3

Schematic representation of main pathways involved in PCH. A. Schematic and simplified representation of tRNA splicing by the TSEN complex and CLP1. B. Schematic figure of the exosome complex including EXOSC3, located in the RNA binding cap, and EXOSC8, located in the core ring. The exosome complex processes mRNA, rRNA and presumably tRNA. C. Upper: charging of tRNA-Arg by RARS2. Lower: conversion of Sec-tRNA-Ser to Sec-tRNA-Sec by SEPSECS

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