Noemie Remacle
Overview
Explore the profile of Noemie Remacle including associated specialties, affiliations and a list of published articles.
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Articles
6
Citations
58
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0
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Recent Articles
1.
Gonzalez Melo M, Remacle N, Cudre-Cung H, Roux C, Poms M, Cudalbu C, et al.
Mol Genet Metab
. 2021 May;
133(2):157-181.
PMID: 33965309
Glutaric aciduria type I (GA-I, OMIM # 231670) is an inborn error of metabolism caused by a deficiency of glutaryl-CoA dehydrogenase (GCDH). Patients develop acute encephalopathic crises (AEC) with striatal...
2.
Cudre-Cung H, Remacle N, do Vale-Pereira S, Gonzalez M, Henry H, Ivanisevic J, et al.
Mol Genet Metab
. 2019 Jan;
126(4):416-428.
PMID: 30686684
Glutaric Aciduria type I (GA-I) is caused by mutations in the GCDH gene. Its deficiency results in accumulation of the key metabolites glutaric acid (GA) and 3-hydroxyglutaric acid (3-OHGA) in...
3.
Remacle N, Forny P, Cudre-Cung H, Gonzalez-Melo M, do Vale-Pereira S, Henry H, et al.
Mol Genet Metab
. 2018 Jun;
124(4):266-277.
PMID: 29934063
Background: Methylmalonic aciduria (MMAuria) is an inborn error of metabolism leading to neurological deterioration. In this study, we used 3D organotypic brain cell cultures derived from embryos of a brain-specific...
4.
Braissant O, Jafari P, Remacle N, Cudre-Cung H, Do Vale Pereira S, Ballhausen D
Neuroscience
. 2016 Dec;
343:355-363.
PMID: 27984186
Glutaryl-CoA dehydrogenase (GCDH) is a mitochondrial enzyme that is involved in the degradation of tryptophan, lysine and hydroxylysine. Deficient enzyme activity leads to glutaric aciduria type-I (GA-I). This neurometabolic disease...
5.
Cudre-Cung H, Zavadakova P, do Vale-Pereira S, Remacle N, Henry H, Ivanisevic J, et al.
Mol Genet Metab
. 2016 Sep;
119(1-2):57-67.
PMID: 27599447
Using 3D organotypic rat brain cell cultures in aggregates we recently identified 2-methylcitrate (2-MCA) as the main toxic metabolite for developing brain cells in methylmalonic aciduria. Exposure to 2-MCA triggered...
6.
Wanet A, Remacle N, Najar M, Sokal E, Arnould T, Najimi M, et al.
Int J Biochem Cell Biol
. 2014 Aug;
54:174-85.
PMID: 25084555
Mitochondrial biogenesis and metabolism have recently emerged as important actors of stemness and differentiation. On the one hand, the differentiation of stem cells is associated with an induction of mitochondrial...