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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | Steinkellner, Hannes Scheiber-Mojdehkar, Barbara Goldenberg, Hans Sturm, Brigitte |
| Description | Country affiliation: Austria Author Affiliation: Steinkellner H ( Medical University of Vienna, Department of Medical Chemistry, Waehringerstr. 10, 1090 Vienna, Austria. hannes.steinkellner@meduniwien.ac.at) |
| Abstract | Friedreich's ataxia (FRDA) is an autosomal recessive neurodegenerative disease affecting 1 in 50,000 people and is caused by a GAA-trinucleotide expansion in the frataxin gene located on chromosome locus 9q13 which results in a markedly reduced expression of frataxin, a small mitochondrial protein. The exact function of frataxin is still unknown and currently there is no approved treatment available. In the near future there will be a high demand for measuring frataxin protein levels due to the development of therapeutic strategies for FRDA based on manipulating frataxin expression levels in vivo. In this paper we describe the development of an electrochemiluminescence assay (ECLIA) to measure frataxin protein levels in a 96-well plate format. The ECLIA for frataxin is able to measure human and mouse samples and is highly quantitative, accurate and reproducible, with low intra- and inter-assay error throughout a wide working range. The assay has an excellent precision and provides a new tool for the set up of high-throughput screening for basic research and for clinical studies with FRDA patients. |
| File Format | HTM / HTML |
| ISSN | 00032670 |
| Issue Number | 1-2 |
| Volume Number | 659 |
| e-ISSN | 18734324 |
| Journal | Analytica Chimica Acta |
| Language | English |
| Publisher | Elsevier |
| Publisher Date | 2010-02-05 |
| Publisher Place | Netherlands |
| Access Restriction | One Nation One Subscription (ONOS) |
| Subject Keyword | Discipline Analytical Discipline Chemistry Enzyme-linked Immunosorbent Assay Methods Iron-binding Proteins Analysis Luminescent Measurements Animals Cell Line, Tumor Friedreich Ataxia Diagnosis Metabolism Hela Cells High-throughput Screening Assays Humans K562 Cells Mice Journal Article |
| Content Type | Text |
| Resource Type | Article |
| Subject | Spectroscopy Environmental Chemistry Analytical Chemistry Biochemistry |
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