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ProteomicsDefective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome
Leigh syndrome (LS) is a severe manifestation of mitochondrial disease in children and is currently incurable. The lack of effective models hampers our understanding of the mechanisms underlying the neuronal pathology of LS. Using patient-derived induced pluripotent stem cells and CRISPR/Cas9 engine...
ORGANISM(S): Homo sapiens (Human) 
2021-01-29 | PXD019112 | Pride
Mitochondrial disease  leigh syndrome  ipscs  npcs  brain organoids  surf1 
UnknownLeigh syndrome associated with TRMU gene mutations.
Not available
S-EPMC7749400 | biostudies-literature
UnknownTherapeutic effects of the mitochondrial ROS-redox modulator KH176 in a mammalian model of Leigh Disease.
Not available
S-EPMC5601915 | biostudies-literature
UnknownReanalysis of Exome Data Identifies Novel SLC25A46 Variants Associated with Leigh Syndrome.
Not available
S-EPMC8703603 | biostudies-literature
ModelsRichards2016 - Genome-scale metabolic reconstruction of Methanococcus maripaludis (iMR539)
Richards2016 - Genome-scale metabolic reconstruction of Methanococcus maripaludis (iMR539) This model is described in the article: Exploring Hydrogenotrophic Methanogenesis: a Genome Scale Metabolic Reconstruction of Methanococcus maripaludis. Richards MA, Lie TJ, Zhang J, R...
2024-10-15 | BIOMD0000001099 | BioModels
FROG 
UnknownNDUFB8 Mutations Cause Mitochondrial Complex I Deficiency in Individuals with Leigh-like Encephalomyopathy.
Not available
S-EPMC5985356 | biostudies-literature
UnknownThe hyaluronic acid capsule of Streptococcus uberis is not required for the development of infection and clinical mastitis.
Not available
S-EPMC143150 | biostudies-literature
UnknownDefective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome.
Not available
S-EPMC7997884 | biostudies-literature
UnknownProgressive cerebellar atrophy in a patient with complex II and III deficiency and a novel deleterious variant in SDHA: A Counseling Conundrum.
Not available
S-EPMC8222855 | biostudies-literature
UnknownA Paradox in Bacterial Pathogenesis: Activation of the Local Macrophage Inflammasome Is Required for Virulence of <i>Streptococcus uberis</i>.
Not available
S-EPMC7768481 | biostudies-literature
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