Surfeit locus protein 1

Definition:

Component of the MITRAC (mitochondrial translation regulation assembly intermediate of cytochrome c oxidase complex) complex, that regulates cytochrome c oxidase assembly.

References:

[1]. Andoni Echaniz-Laguna, et al. SURF1 deficiency causes demyelinating Charcot-Marie-Tooth disease. Neurology. 2013 Oct 22;81(17):1523-30. [Content Brief]

[2]. Z Zhu, et al. SURF1, encoding a factor involved in the biogenesis of cytochrome c oxidase, is mutated in Leigh syndrome. Nat Genet. 1998 Dec;20(4):337-43. [Content Brief]

[3]. Sven Dennerlein, et al. MITRAC7 Acts as a COX1-Specific Chaperone and Reveals a Checkpoint during Cytochrome c Oxidase Assembly. Cell Rep. 2015 Sep 8;12(10):1644-55. [Content Brief]

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