نتایج جستجو برای: canavan disease

تعداد نتایج: 1490117  

Journal: :Clinical biochemistry 2005
Barbara Tavazzi Giuseppe Lazzarino Paola Leone Angela Maria Amorini Francesco Bellia Christopher G Janson Valentina Di Pietro Lia Ceccarelli Sonia Donzelli Jeremy S Francis Bruno Giardina

OBJECTIVES To set up a novel simple, sensitive, and reliable ion-pairing HPLC method for the synchronous separation of several purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis and screening of inborn errors of metabolism (IEM). DESIGN AND METHODS The separation was set up using a Hypersil C-18, 5-microm particle size, 250 x 4.6 mm column, and a...

Journal: :Journal of visualized experiments : JoVE 2016
Sarah Jolly Alexander Fudge Nigel Pringle William D Richardson Huiliang Li

Detection of gene expression in different types of brain cells e.g., neurons, astrocytes, oligodendrocytes, oligodendrocyte precursors and microglia, can be hampered by the lack of specific primary or secondary antibodies for immunostaining. Here we describe a protocol to detect the expression of three different genes in the same brain section using double fluorescence in situ hybridization wit...

Canavan disease is an autosomal recessive leukodystrophy due to accumulation of N-acetyl aspartic acid (NAA) in brain, cerebrospinal fluid (CSF), and urine characterized by early onset developmental delay, initial hypotonia progressing to hypertonia, sleep disturbance and macrocephaly. Brain magnetic resonance imaging (MRI) shows white-matter changes. The best method for diagnosis is determined...

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