Protein Information

Name FRDA
Synonyms CyaY; FA; FARR; FRDA; FXN; Frataxin; Friedreich ataxia protein; X25…

Compound Information

Name rotenone
CAS

Reference List

PubMed Abstract RScore(About this table)
16815102 Cortopassi G, Danielson S, Alemi M, Zhan SS, Tong W, Carelli V, Martinuzzi A, Marzuki S, Majamaa K, Wong A: Mitochondrial disease activates transcripts of the unfolded protein response and cell cycle and inhibits vesicular secretion and oligodendrocyte-specific transcripts. Mitochondrion. 2006 Aug;6(4):161-75. Epub 2006 May 24.

To examine the extent to which the transcriptional profile was shared among 5 mitochondrial diseases (LHON, FRDA, MELAS, KSS, and NARP), we microarrayed mutant and control groups in N-tera2, SH-SY5Y, lymphoblasts, fibroblasts, myoblasts, muscle, and osteosarcoma cybrids.
Mitochondrial disease activated multiple transcripts of the unfolded protein response (UPR), and of the cell cycle pathway, and low doses of the mitochondrial inhibitor rotenone induced UPR transcripts in the absence of cell death.
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