Disruption of Lrpprc affects B cell development and proliferation in a mouse model of Leigh Syndrome French Canadian type

Disruption of Lrpprc affects B cell development and proliferation in a mouse model of Leigh Syndrome French Canadian type

Abstract Purpose Leigh Syndrome French Canadian (LSFC) is a rare autosomal recessive metabolic disorder characterized by severe lactic acidosis crises and early mortality. LSFC patients carry variants in the Leucine Rich Pentatricopeptide Repeat Containing (LRPPRC) nuclear gene, which lead to defect...

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Main Authors: Adrien Fois, Sonia Deschênes, Capucine Bourel, Claudine Beauchamp, Félix Lombard-Vadnais, Matthieu Ruiz, Guy Charron, Lise Coderre, LSFC Consortium, John D. Rioux, Sylvie Lesage
Format: Article
Language:English
Published: Springer 2025-07-01
Series:Journal of Rare Diseases
Subjects:
LSFC
LRPPRC
Immune cells
Mitochondria
Proliferation
B cells
Online Access:https://doi.org/10.1007/s44162-025-00094-x
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https://doi.org/10.1007/s44162-025-00094-x

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