Childhood Ataxia: Clinical Features, Pathogenesis, Key Unanswered Questions, and Future Directions.

Childhood Ataxia: Clinical Features, Pathogenesis, Key Unanswered Questions, and Future Directions. Ashley CN, Hoang KD, Lynch DR, Perlman SL, Maria BL.J Child Neurol 0883073812448840, first published on August 1, 2012 Keywords: Childhood ataxia, Friedreich ataxia, GAA repeat expansion, frataxin gene, mitochondrial function, reactive oxygen species, iron.

The p53-depen dent expression of frataxin controls 5-aminolevulinic acid (ALA)-induced accumulation of protoporphyrin IX and photo-damage in cancerous cells

The p53-depen dent expression of frataxin controls 5-aminolevulinic acid (ALA)-induced accumulation of protoporphyrin IX and photo-damage in cancerous cells. Mari Sawamoto, Takafumi Imai, Mana Umeda, Koji Fukuda, Takao Kataoka and Shigeru Taketani; Photochemistry and Photobiology. Accepted manuscript online: 3 AUG 2012 02:40AM EST | DOI: 10.1111/j.1751-1097.2012.01215.x Keyword: Mitochondrial frataxin, iron homeostasis, iron-sulfur cluster biogenesis, oxidative stress, apoptosis, tumor suppressor, p53-dependent, heme biosynthesis, 5-aminolevulinic acid (ALA).