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Mendeley readers
| Chapter title |
Conditional mouse models for Friedreich ataxia, a neurodegenerative disorder associating cardiomyopathy.
|
|---|---|
| Chapter number | 15 |
| Book title |
Conditional Mutagenesis: An Approach to Disease Models
|
| Published in |
Handbook of experimental pharmacology, January 2007
|
| DOI | 10.1007/978-3-540-35109-2_15 |
| Pubmed ID | |
| Book ISBNs |
978-3-54-035108-5, 978-3-54-035109-2
|
| Authors |
H. Puccio |
| Abstract |
Friedreich ataxia (FRDA), the most common recessive ataxia, is characterized by degeneration of the large sensory neurons and spinocerebellar tracts and cardiomyopathy. It is caused by severely reduced levels of frataxin, a mitochondrial protein involved in iron-sulfur cluster (ISC) biosynthesis. Mouse models have been important tools in dissecting the steps of pathogenesis in FRDA. Furthermore, animal models that reproduce some of the key events in a pathology are essential for the development of effective therapies, both pharmacological and gene therapy approaches. This chapter presents an overview of the current mouse models that have been developed for FRDA. |
Mendeley readers
The data shown below were compiled from readership statistics for 32 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Italy | 1 | 3% |
| Unknown | 31 | 97% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Ph. D. Student | 8 | 25% |
| Researcher | 7 | 22% |
| Student > Doctoral Student | 3 | 9% |
| Other | 3 | 9% |
| Professor | 2 | 6% |
| Other | 5 | 16% |
| Unknown | 4 | 13% |
| Readers by discipline | Count | As % |
|---|---|---|
| Agricultural and Biological Sciences | 15 | 47% |
| Biochemistry, Genetics and Molecular Biology | 6 | 19% |
| Neuroscience | 3 | 9% |
| Chemistry | 2 | 6% |
| Medicine and Dentistry | 1 | 3% |
| Other | 1 | 3% |
| Unknown | 4 | 13% |