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phosphorylase/seizures

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The aim of the present study was to examine the total, as well as the active form of glycogen phosphorylase in the rat cerebral cortex during development, and to assess the response of the enzyme to induced seizures. Seizures were induced in 7-, 12- and 18-day-old male Wistar rats by i.p.

Changes of cyclic AMP and phosphorylase a in mouse cerebral cortex during seizures induced by 3-mercaptopropionic acid.

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Altered Plasticity of Glycogen Phosphorylase in Forebrain Gliosomes Obtained from Insulinoma Patients.

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We investigated a control model of hypoglycemia-exposed brain tissues from a small series of patients with insulinoma, immediately dissect them, and perform a differential cold centrifugation to obtain gliosomes and examine alterations of glycogenolytic mechanisms. The BB as well as MM isoforms of
Glial cells provide energy substrates to neurons, in part from glycogen metabolism, which is influenced by glycogen phosphorylase (GP). To gain insight into the potential subfield and laminar-specific expression of GP, histochemistry can be used to evaluate active GP (GPa) or totalGP (GPa + GPb).

Convulsions as the etiology of lactic acidosis in acute diazinon toxicity in rats.

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Diazinon, in acute doses (40 mg/kg, i.p.) in rats produced tremors and convulsions with lactic acidosis which was accompanied by depletion of glycogen and activation of glycogen phosphorylase activity in triceps and diaphragm muscles, 2 h after its administration. Prevention of convulsions with

Purine nucleoside phosphorylase deficiency with a novel PNP gene mutation: a first case report from India.

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The authors report a case of purine nucleoside phosphorylase (PNP) deficiency for the first time from India. The case presented with recurrent severe infections, developmental delays, seizures and progressive neurological deterioration. The diagnosis of primary immunodeficiency disorder was delayed

[Acute renal failure due to rhabdomyolysis in McArdle's disease following binge drinking with seizures].

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METHODS A 36-year-old gardener was admitted for tonic-clonic seizures after binge drinking. The next days he developed massive rhabdomyolysis with acute renal failure. Past medical history was unremarkable except for a similar episode of acute renal failure 14 years ago. At that time he had consumed
X-linked liver glycogenosis (XLG) resulting from phosphorylase kinase (Phk) deficiency is one of the most common forms of glycogen storage disease. It is caused by mutations in the gene encoding the liver isoform of the Phk alpha subunit (PHKA2). In the present study, we address the issue of

[Hepatic glycogenosis in childhood: clinical and laboratory findings in 20 patients].

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We studied 20 children with a clinical picture and laboratory study suggestive of hepatic glycogenosis. The age of the beginning of symptoms varied from birth to 24 months and the age at the diagnosis varied from 2 to 81 months. Hepatomegaly was found in all patients, diarrhea in 65% (13/26),

Levetiracetam in children with refractory epilepsy: a multicenter open label study in Germany.

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OBJECTIVE To evaluate the efficacy and tolerability of Levetiracetam (LEV) in a large pediatric cohort with drug-resistant epilepsy from a prospective multicenter observational study. METHODS We report the results of a multicenter observational survey of a cohort of 285 pediatric patients (mean: 9.9

High glycogen levels in the hippocampus of patients with epilepsy.

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During intense cerebral activation approximately half of the glucose plus lactate taken up by the human brain is not oxidized and could replenish glycogen deposits, but the human brain glycogen concentration is unknown. In patients with temporal lobe epilepsy, undergoing curative surgery, brain

Laforin prevents stress-induced polyglucosan body formation and Lafora disease progression in neurons.

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Glycogen, the largest cytosolic macromolecule, is soluble because of intricate construction generating perfect hydrophilic-surfaced spheres. Little is known about neuronal glycogen function and metabolism, though progress is accruing through the neurodegenerative epilepsy Lafora disease (LD)
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