CoA‐dependent activation of mitochondrial acyl carrier protein links four neurodegenerative diseases

CoA‐dependent activation of mitochondrial acyl carrier protein links four neurodegenerative diseases

Abstract PKAN, CoPAN, MePAN, and PDH‐E2 deficiency share key phenotypic features but harbor defects in distinct metabolic processes. Selective damage to the globus pallidus occurs in these genetic neurodegenerative diseases, which arise from defects in CoA biosynthesis (PKAN, CoPAN), protein lipoyla...

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Main Authors: Roald A Lambrechts, Hein Schepers, Yi Yu, Marianne van der Zwaag, Kaija J Autio, Marcel A Vieira‐Lara, Barbara M Bakker, Marina A Tijssen, Susan J Hayflick, Nicola A Grzeschik, Ody CM Sibon
Format: Article
Language:English
Published: Springer Nature 2019-11-01
Series:EMBO Molecular Medicine
Subjects:
4′‐phosphopantetheinylation
Coenzyme A
mtACP
NBIA
NDUFAB1
Online Access:https://doi.org/10.15252/emmm.201910488
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https://doi.org/10.15252/emmm.201910488

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