leigh disease/hypoxia

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Subacute necrotizing encephalomyelopathy (Leigh's disease): clinical correlations with computerized tomography in the diagnosis of the juvenile and adult forms.

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Ingia / Ingia

A computerized tomography (CT) scan was performed on 6 surviving patients with a suspected diagnosis of subacute necrotizing encephalomyelopathy of the juvenile and adult forms. Bilateral low density areas were observed in the putamina in all cases. This CT pattern conformed with the characteristic

Fatal breathing dysfunction in a mouse model of Leigh syndrome.

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Ingia / Ingia

Leigh syndrome (LS) is a subacute necrotizing encephalomyelopathy with gliosis in several brain regions that usually results in infantile death. Loss of murine Ndufs4, which encodes NADH dehydrogenase (ubiquinone) iron-sulfur protein 4, results in compromised activity of mitochondrial complex I as

Hypoxic and hypercapnic challenges unveil respiratory vulnerability of Surf1 knockout mice, an animal model of Leigh syndrome.

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Ingia / Ingia

Surf1 gene mutations were detected as a main cause for Leigh syndrome (LS), also known as infantile subacute necrotizing encephalomyelopathy. This syndrome which is commonly associated with systemic cytochrome c oxidase (COX) deficiency manifests in early childhood and has an invariable poor

Lactic acidosis in childhood.

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Ingia / Ingia

Children with chronic metabolic acidosis should be investigated to determine the presence of an organic acid, especially when the plasma electrolyte profile shows a deficiency of anion. One of the organic acids that should be looked for in such a patient is lactic acid. Lactic acidosis due to tissue

Mitochondrial retrograde signaling regulates neuronal function.

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Ingia / Ingia

Mitochondria are key regulators of cellular homeostasis, and mitochondrial dysfunction is strongly linked to neurodegenerative diseases, including Alzheimer's and Parkinson's. Mitochondria communicate their bioenergetic status to the cell via mitochondrial retrograde signaling. To investigate the

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Herbal & Natural Medicine

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