نتایج جستجو برای: tay sachs disease

تعداد نتایج: 1492516  

Journal: :The Journal of molecular diagnostics : JMD 2009
Lisa Kalman Jean Amos Wilson Arlene Buller John Dixon Lisa Edelmann Louis Geller William Edward Highsmith Leonard Holtegaard Ruth Kornreich Elizabeth M Rohlfs Toby L Payeur Tina Sellers Lorraine Toji Kasinathan Muralidharan

Many recessive genetic disorders are found at a higher incidence in people of Ashkenazi Jewish (AJ) descent than in the general population. The American College of Medical Genetics and the American College of Obstetricians and Gynecologists have recommended that individuals of AJ descent undergo carrier screening for Tay Sachs disease, Canavan disease, familial dysautonomia, mucolipidosis IV, N...

Journal: :Journal of Clinical Investigation 1968

Journal: :Archives of Physical Medicine and Rehabilitation 1994

Journal: :The Journal of clinical investigation 1960
E G TRAMS R O BRADY

Familial lipodystrophic conditions, such as Gaucher's, Niemann-Pick, and Tay-Sachs disease, are characterized by the intracellular accumulation of abnormally large quantities of sphingolipids. In Gaucher's disease, the offending lipids are cerebrosides, while Niemann-Pick and Tay-Sachs diseases are characterized by the accumulation of sphingomyelin and gangliosides, respectively. In Gaucher's a...

2012
Timothy J. Sargeant Deborah J. Drage Susan Wang Apostolos A. Apostolakis Timothy M. Cox M. Begoña Cachón-González

Tay-Sachs and Sandhoff diseases are lethal inborn errors of acid β-N-acetylhexosaminidase activity, characterized by lysosomal storage of GM2 ganglioside and related glycoconjugates in the nervous system. The molecular events that lead to irreversible neuronal injury accompanied by gliosis are unknown; but gene transfer, when undertaken before neurological signs are manifest, effectively rescue...

2013

Familial lipodystrophic conditions, such as Gaucher's, Niemann-Pick, and Tay-Sachs disease, are characterized by the intracellular accumulation of abnormally large quantities of sphingolipids. In Gaucher's disease, the offending lipids are cerebrosides, while Niemann-Pick and Tay-Sachs diseases are characterized by the accumulation of sphingomyelin and gangliosides, respectively. In Gaucher's a...

Journal: :Arquivos De Neuro-psiquiatria 2023

Case presentation: Boy, 4 years old. Born term, cesarean for oligohydramnios, without consanguinity. Mother with hypothyroidism, maternal uncle autism, cousin epilepsy and paternal cerebral palsy. Adequate neuropsychomotor up to 2 of age. At this age, started ataxic gait, refractory epilepsy, spasticity, language loss dysphagia. Multiple hospitalizations due bronchoaspiration pneumonia. Gastros...

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