Metallothioneins as dynamic markers for brain disease in lysosomal disorders

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Metallothioneins as dynamic markers for brain disease in lysosomal disorders

OBJECTIVE To facilitate development of novel disease-modifying therapies for lysosomal storage disorder (LSDs) characterized by nervous system involvement such as metachromatic leukodystrophy (MLD), molecular markers for monitoring disease progression and therapeutic response are needed. To this end, we sought to identify blood transcripts associated with the progression of MLD. METHODS Genom...

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how to cite this article: shakiba m. diagnosis in lysosomal disorders. iran j child neurol autumn 2012; 6:4 (suppl. 1):15- 16. pls see pdf.

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Biomarkers for lysosomal storage disorders: identification and application as exemplified by chitotriosidase in Gaucher disease.

UNLABELLED A biomarker is an analyte that indicates the presence of a biological process linked to the clinical manifestations and outcome of a particular disease. An ideal biomarker provides indirect but ongoing determinations of disease activity. In the case of lysosomal storage disorders (LSDs), metabolites or proteins specifically secreted by storage cells are good candidates for biomarkers...

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Disease-specific, neurosphere-derived cells as models for brain disorders.

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Role of metallothioneins in disease.

Metallothioneins are small proteins (6,000 to 10,000 mw) with similar amino acid compositions, high content of sulfhydryl amino acids and no aromatic amino acids. Emax at 250 nm is due to cadmium mercaptide bond. Synthesis is induced by a number of metals including zinc, cadmium, mercury, copper, bismuth, gold and silver. These proteins function as a mechanism of intracellular storage of some e...

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ژورنال

عنوان ژورنال: Annals of Neurology

سال: 2014

ISSN: 0364-5134,1531-8249

DOI: 10.1002/ana.24053