A Chemical Biology Approach to Model Pontocerebellar Hypoplasia Type 1B (PCH1B).

doi:10.1021/acschembio.8b00745

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A Chemical Biology Approach to Model Pontocerebellar Hypoplasia Type 1B (PCH1B).

ACS chemical biology - 19 Oct 2018

François-Moutal Liberty, Jahanbakhsh Shahriyar, Nelson Andrew D L, Ray Debashish, Scott David D, Hennefarth Matthew R, Moutal Aubin, Perez-Miller Samantha, Ambrose Andrew J, Al-Shamari Ahmed, Coursodon Philippe, Meechoovet Bessie, Reiman Rebecca, Lyons Eric, Beilstein Mark, Chapman Eli, Morris Quaid D, Van Keuren-Jensen Kendall, Hughes Timothy R, Khanna Rajesh, Koehler Carla, Jen Joanna, Gokhale Vijay, Khanna May

Abstract excerpt

Mutations of EXOSC3 have been linked to the rare neurological disorder known as Pontocerebellar Hypoplasia type 1B (PCH1B). EXOSC3 is one of three putative RNA-binding structural cap proteins that guide RNA into the RNA exosome, the cellular machinery that degrades RNA. Using RNAcompete, we identified a G-rich RNA motif binding to EXOSC3. Surface plasmon resonance (SPR) and microscale thermophoresis (MST)...

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Identifiers

PMID: 30141626
DOI: 10.1021/acschembio.8b00745
PMCID: PMC6504997

Genes

EXOSC3
SPR

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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