Frataxin overexpressing mice

• Type: Article in International Scientific Journal
• Date: 2004

Authors

Miranda, CJ (Author) Other; Santos, MM (Author) REIT; Ohshima, K (Author) Other; Tessaro, M (Author) Other; Sequeiros, J (Author) ICBAS; Pandolfo, M (Author) Other

Journal

Title: FEBS Letters

Vol. 572

Pages: 281-288

ISSN: 0014-5793

Publisher: Wiley-Blackwell

Indexing

ISI Web of Knowledge - 23 Citations

Scopus - 24 Citations

Other information

Authenticus ID: P-000-90E

DOI: 10.1016/j.febslet.2004.07.022

Abstract (EN): Friedreich ataxia, the most common autosomal recessive ataxia, is caused by frataxin deficiency. Reduction of frataxin has been associated with iron accumulation and sensitivity to iron induced oxidative stress. To better understand the function of frataxin, transgenic mice (tgFxn) overexpressing human frataxin were generated. Iron metabolism parameters in tgFxn were normal and no signs of ataxia or other obvious abnormalities were observed, indicating that overexpression of frataxin in mouse is innocuous. Several hypotheses for frataxin function were evaluated in tgFxn mice. In particular, we observed that TgFxn mice show an altered response during hematopoietic differentiation, suggesting that frataxin may directly affect heme synthesis.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 8