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Старонка 1 ад 28 вынікі
Light at the End of the "TUNEL"? Role of Ceramide in Seizure-induced Programmed Cell Death.
Перакладаць артыкулы могуць толькі зарэгістраваныя карыстальнікі
Увайсці / Зарэгістравацца
Mechanism of action of antiepileptic ceramide from Red Sea soft coral Sarcophyton auritum.
Перакладаць артыкулы могуць толькі зарэгістраваныя карыстальнікі
Увайсці / Зарэгістравацца
Chemical investigation of the Red Sea soft coral Sarcophyton auritum led to the isolation and structure elucidation of a new ceramide N-((2S,3R,4E,6E)-1,3-dihydroxyhenicosa-4,6-dien-2-yl)tridecanamide (1). Structure elucidation was achieved using spectroscopic techniques, including 1D and 2D NMR and
Autosomal recessive progressive myoclonus epilepsy due to impaired ceramide synthesis.
Перакладаць артыкулы могуць толькі зарэгістраваныя карыстальнікі
Увайсці / Зарэгістравацца
Autosomal recessive progressive myoclonus epilepsy due to impaired ceramide synthesis is an extremely rare condition, so far reported in a single family of Algerian origin presenting an unusual, severe form of progressive myoclonus epilepsy characterized by myoclonus, generalized tonic-clonic
Hippocampal programmed cell death after status epilepticus: evidence for NMDA-receptor and ceramide-mediated mechanisms.
Перакладаць артыкулы могуць толькі зарэгістраваныя карыстальнікі
Увайсці / Зарэгістравацца
OBJECTIVE
Status epilepticus (SE) can result in acute neuronal injury with subsequent long-term age-dependent behavioral and histologic sequelae. To investigate potential mechanisms that may underlie SE-related neuronal injury, we studied the occurrence of programmed cell death (PCD) in the
Upregulation of Bcl-2 and elevation of ceramide in Batten disease.
Перакладаць артыкулы могуць толькі зарэгістраваныя карыстальнікі
Увайсці / Зарэгістравацца
The late infantile and juvenile variants of Batten disease are genetically distinct neurodegenerative disorders. Hallmarks of Batten disease include cognitive and motor decline, seizures and blindness due to retinitis pigmentosa. Recently, the CLN3 gene responsible for the juvenile variant has been
Evaluation of sphingolipids changes in brain tissues of rats with pentylenetetrazol-induced kindled seizures using MALDI-TOF-MS.
Перакладаць артыкулы могуць толькі зарэгістраваныя карыстальнікі
Увайсці / Зарэгістравацца
Abnormal lipid metabolism has been implicated in the pathogenesis of many neural system diseases, including epilepsy. Pentylenetetrazol (PTZ)-induced kindling in rodents is considered a model of human absence epilepsy and myoclonic, generalized tonic-clonic seizure. In an effort to further
CLN3 defines a novel antiapoptotic pathway operative in neurodegeneration and mediated by ceramide.
Перакладаць артыкулы могуць толькі зарэгістраваныя карыстальнікі
Увайсці / Зарэгістравацца
Juvenile neuronal ceroid lipofuscinosis or Batten disease (JNCL) is a neurodegenerative disorder characterized by blindness, seizures, cognitive decline and early death. Brain atrophy and retinitis pigmentosa ensue because of neuronal and photoreceptor apoptosis. The CLN3 gene defective in JNCL
Elevated sulfatide levels in neurons cause lethal audiogenic seizures in mice.
Перакладаць артыкулы могуць толькі зарэгістраваныя карыстальнікі
Увайсці / Зарэгістравацца
Galactosylceramide (GalCer) and 3-O-sulfo-GalCer (sulfatide) are abundant sphingolipids in myelinating glial cells. However, low levels of GalCer and sulfatide have also been found in neurons, though their physiological role in these cells is unknown. Transgenic mice over-expressing UDP-galactose :
Antiepileptic ceramides from the Red Sea sponge Negombata corticata.
Перакладаць артыкулы могуць толькі зарэгістраваныя карыстальнікі
Увайсці / Зарэгістравацца
A new antiepileptic ceramide mixture 1 was isolated from the Red Sea sponge Negombata corticata. The structures of the metabolites were determined by extensive spectroscopic analysis. The anticonvulsant activity of 1 was measured in vivo using the pentylenetetrazole-induced seizure model. This
Farber lipogranulomatosis type 1--late presentation and early death in a Croatian boy with a novel homozygous ASAH1 mutation.
Перакладаць артыкулы могуць толькі зарэгістраваныя карыстальнікі
Увайсці / Зарэгістравацца
BACKGROUND
We report a boy with an unusually late presentation of Farber lipogranulomatosis type l.
METHODS
The first symptoms appeared at the end of the first year of life in the form of joint swelling; other symptoms such as cherry-red spot, hoarseness, subcutaneous nodules appeared much later.
Clinical, pathological, and biochemical studies on an infantile case of sulfatide/GM1 activator protein deficiency.
Перакладаць артыкулы могуць толькі зарэгістраваныя карыстальнікі
Увайсці / Зарэгістравацца
A 28-month-old black male died with severe complications of mental and motor deterioration, seizures, and aspiration. Autopsy demonstrated moderate liver enlargement, normal spleen and kidneys, small testes, and a grossly normal brain. Further examination showed irregular macrogyrae with evidence of
Pharmacological Effects on Ceroid Lipofuscin and Neuronal Structure in Cln3 ∆ex7/8 Mouse Brain Cultures.
Перакладаць артыкулы могуць толькі зарэгістраваныя карыстальнікі
Увайсці / Зарэгістравацца
Juvenile Batten disease (JBD) is an inherited disorder that is characterized by the development of blindness, seizures, and progressive motor, psychiatric, and cognitive impairment. A model of JBD expressing the predominant human mutation (Cln3 ∆ex7/8 ) has been explored. Dissociated brain cultures
Clinical, pathologic, and neurochemical studies of an unusual case of neuronal storage disease with lamellar cytoplasmic inclusions: a new genetic disorder?
Перакладаць артыкулы могуць толькі зарэгістраваныя карыстальнікі
Увайсці / Зарэгістравацца
A child of first-cousin Puerto Rican parents had global developmental delay, failure to thrive, and hypotonia since early infancy. At 1 1/2 years of age, she developed clinical and electrophysiologic evidence of progressive motor and sensory neuropathy. At 2 1/2 years, she developed visual
Defective cortex glia plasma membrane structure underlies light-induced epilepsy in cpes mutants.
Перакладаць артыкулы могуць толькі зарэгістраваныя карыстальнікі
Увайсці / Зарэгістравацца
Seizures induced by visual stimulation (photosensitive epilepsy; PSE) represent a common type of epilepsy in humans, but the molecular mechanisms and genetic drivers underlying PSE remain unknown, and no good genetic animal models have been identified as yet. Here, we show an animal model of PSE, in
Neuronopathic juvenile glucosylceramidosis due to sap-C deficiency: clinical course, neuropathology and brain lipid composition in this Gaucher disease variant.
Перакладаць артыкулы могуць толькі зарэгістраваныя карыстальнікі
Увайсці / Зарэгістравацца
Glucosylceramide lipidosis results from a defective lysosomal degradation of this glycolipid. Lipid degradation is controlled by two components, the enzyme beta-glucocerebrosidase and a sphingolipid activator protein. While most Gaucher cases are due to mutations within the gene that codes for the
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