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Updated: Sep 10, 2025

Rapid Isolation of the Mitoribosome from HEK Cells
Published on: October 4, 2018
Is mitochondrial function at the heart of ribosome-related diseases?
Qiuxia Zhao1, Elif Sarinay Cenik1
1Department of Molecular Biosciences, University of Texas at Austin, Austin, TX, USA.
Abstract:
Defects in ribosomal machinery cause ribosomopathies such as Diamond Blackfan anemia, classically linked to impaired protein synthesis. However, emerging evidence places mitochondrial dysfunction as a critical downstream consequence of ribosomal insufficiency. Thus, is impaired energy metabolism, rather than translation alone, a key driver of ribosomopathies such as Diamond Blackfan anemia? This insight could reframe our understanding of disease mechanisms and could identify metabolic pathways as promising therapeutic targets.
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