Redução na concentração de glicose no cérebro de camundongos inoculados com liquido cefalorraquiano de pacientes com esclerose lateral amiotrófica

Godoy, J. M.; Skacel, M.; Lima, J.A. De; Andrade, Carolina M. M. C.

doi:10.1590/s0004-282x1990000400012

Cited by 1 publication

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References 19 publications

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“…Glucose utilization (35) and monocarboxylate transporter 1 (MCT1)-mediated lactate uptake (36) within the CNS are inhibited by acidosis. Indeed, glucose uptake and/or levels within the CNS are significantly diminished in patients with ALS (37), SOD1 G93A mice (32,38), and in WT mice exposed to cerebrospinal fluid (CSF) from patients with ALS (39). Moreover, lactate levels are reduced in spinal cords of SOD1 G93A mice (19), as is the expression of MCT1 (36).…”

Section: Discussionmentioning

confidence: 99%

Metabolic signatures of amyotrophic lateral sclerosis reveal insights into disease pathogenesis

Dodge¹,

Treleaven²,

Fidler³

et al. 2013

Proc. Natl. Acad. Sci. U.S.A.

110

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Metabolic dysfunction is an important modulator of disease course in amyotrophic lateral sclerosis (ALS). We report here that a familial mouse model (transgenic mice over-expressing the G93A mutation of the Cu/Zn superoxide dismutase 1 gene) of ALS enters a progressive state of acidosis that is associated with several metabolic (hormonal) alternations that favor lipolysis. Extensive investigation of the major determinants of H + concentration (i.e., the strong ion difference and the strong ion gap) suggests that acidosis is also due in part to the presence of an unknown anion. Consistent with a compensatory response to avert pathological acidosis, ALS mice harbor increased accumulation of glycogen in CNS and visceral tissues. The altered glycogen is associated with fluctuations in lysosomal and neutral α-glucosidase activities. Disease-related changes in glycogen, glucose, and α-glucosidase activity are also found in spinal cord tissue samples of autopsied patients with ALS. Collectively, these data provide insights into the pathogenesis of ALS as well as potential targets for drug development.

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