E3 Ubiquitin Ligases in Neurological Diseases: Focus on Gigaxonin and Autophagy

Frontiers in Physiology
Léa Lescouzères, Pascale Bomont

Abstract

Ubiquitination is a dynamic post-translational modification that regulates the fate of proteins and therefore modulates a myriad of cellular functions. At the last step of this sophisticated enzymatic cascade, E3 ubiquitin ligases selectively direct ubiquitin attachment to specific substrates. Altogether, the ∼800 distinct E3 ligases, combined to the exquisite variety of ubiquitin chains and types that can be formed at multiple sites on thousands of different substrates confer to ubiquitination versatility and infinite possibilities to control biological functions. E3 ubiquitin ligases have been shown to regulate behaviors of proteins, from their activation, trafficking, subcellular distribution, interaction with other proteins, to their final degradation. Largely known for tagging proteins for their degradation by the proteasome, E3 ligases also direct ubiquitinated proteins and more largely cellular content (organelles, ribosomes, etc.) to destruction by autophagy. This multi-step machinery involves the creation of double membrane autophagosomes in which engulfed material is degraded after fusion with lysosomes. Cooperating in sustaining homeostasis, actors of ubiquitination, proteasome and autophagy pathways are impaired or ...Continue Reading

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Citations

Jan 18, 2021·Current Opinion in Cell Biology·Pascale Bomont
May 3, 2021·Biochemical and Biophysical Research Communications·KyoungJoo ChoSeung Ho Choi
May 25, 2021·Frontiers in Immunology·Sergio Castro-GonzalezRuth Serra-Moreno

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Datasets Mentioned

BETA
AF291673

Methods Mentioned

BETA
ubiquitination
Human Genome sequencing
antisense oligonucleotide
biopsies
glycosylation
gene knock-down
lipidation
ELISA
nucleotide exchange
nuclear translocation

Clinical Trials Mentioned

NCT04259281
NCT02362438

Software Mentioned

CRBN
GAN

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