Endocrinologic Regulation of Carbohydrate Metabolism

Nagano, Y; Tsubaki, Tadao; Chase, Thomas N.

doi:10.1001/archneur.1979.00500400071011

Cited by 12 publications

(1 citation statement)

References 32 publications

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“…Additional evidence of altered carbohydrate metabolism comes from a study of 10 ALS patients from Guam, aged 40–63 [33]. After exposing study participants to intravenous arginine infusion, which stimulates insulin secretion, it was found that post infusion ALS patients showed a slight albeit not significant increase in glucose levels.…”

Section: Carbohydrate Metabolism and Alsmentioning

confidence: 99%

Metabolic Dysregulation in Amyotrophic Lateral Sclerosis: Challenges and Opportunities

2017

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Purpose of Review Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease for which there is no cure and treatments are at best palliative. Several genes have been linked to ALS, which highlight defects in multiple cellular processes including RNA processing, proteostasis and metabolism. Clinical observations have identified glucose intolerance and dyslipidemia as key features of ALS however the causes of these metabolic alterations remain elusive. Recent Findings Recent studies reveal that motor neurons and muscle cells may undergo cell type specific metabolic changes that lead to utilization of alternate fuels. For example, ALS patients’ muscles exhibit reduced glycolysis and increased reliance on fatty acids. In contrast, ALS motor neurons contain damaged mitochondria and exhibit impaired lipid beta oxidation, potentially leading to increased glycolysis as a compensatory mechanism. Summary These findings highlight the complexities of metabolic alterations in ALS and provide new opportunities for designing therapeutic strategies based on restoring cellular energetics.

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Section: Carbohydrate Metabolism and Alsmentioning

confidence: 99%

Metabolic Dysregulation in Amyotrophic Lateral Sclerosis: Challenges and Opportunities

2017

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Amyotrophic leteral sclerosis: Part 2. Etiopathogenesis

Tandan

Bradley

1985

Annals of Neurology

140

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The pathogenesis of the motor neuronal degeneration in amyotrophic lateral sclerosis (ALS) is unclear, though several possible etiological factors are currently being investigated. A unifying hypothesis will have to explain the diverse geographical occurrence, clinical features, and selective vulnerability and relative resistance of different neuronal populations in the disease. It is possible that different biochemical defects underlie this diversity, or alternatively that the many factors incriminated in the etiology may act upon an underlying genetic-biochemical abnormality to trigger premature neuronal death. Viruses, metals, endogenous toxins, immune dysfunction, endocrine abnormalities, impaired DNA repair, altered axonal transport, and trauma have all been etiologically linked with ALS, but convincing research evidence of a causative role for any of these factors is yet to be demonstrated.

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Impact of the metabolic syndrome on prevalence and survival in motor neuron disease: a retrospective case series

Oh,

Demopoulos

et al. 2023

Metab Brain Dis

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Metabolic dysfunction is an important factor in the pathogenesis of motor neuron disease, but its prevalence and association with survival in this disorder is unknown. We hypothesized that patients with motor neuron disease would show a higher prevalence of metabolic syndrome compared to the general New Zealand population, and that metabolic syndrome would be associated with worsened survival. We undertook a retrospective analysis in 109 motor neuron disease patients diagnosed and treated at Waikato Hospital from 2013 to 2020. Demographic, clinical, and laboratory data were collected. Survival was defined as the date of initial symptom onset to the date of death. Of 104 eligible patients, 34 patients (33%) had metabolic syndrome (33% of Europeans, 46% of Māori). Mean survival in motor neuron disease patients with metabolic syndrome was significantly reduced compared to patients without metabolic syndrome (38 vs. 61 months, P = 0.044), with a 5-year survival rate of 21% for the former and 38% for the latter (P = 0.012). Compared with the general New Zealand population, metabolic syndrome is highly prevalent amongst motor neuron disease patients in the Waikato region and it is associated with worsened survival. Metabolic dysfunction may be a key factor underlying the pathogenesis of motor neuron disease.

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Endocrinologic Regulation of Carbohydrate Metabolism

Cited by 12 publications

References 32 publications

Metabolic Dysregulation in Amyotrophic Lateral Sclerosis: Challenges and Opportunities

Metabolic Dysregulation in Amyotrophic Lateral Sclerosis: Challenges and Opportunities

Amyotrophic leteral sclerosis: Part 2. Etiopathogenesis

Impact of the metabolic syndrome on prevalence and survival in motor neuron disease: a retrospective case series

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