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Hwang, DK, Claypool, SM, Leuenberger, D, Tienson, HL and Koehler, CM (2007) Tim54p connects inner membrane assembly and proteolytic pathways in the mitochondrion. J. Cell Biol. 178:1161-75


Tim54p, a component of the inner membrane TIM22 complex, does not directly mediate the import of inner membrane substrates but is required for assembly/stability of the 300-kD TIM22 complex. In addition, Deltatim54 yeast exhibit a petite-negative phenotype (also observed in yeast harboring mutations in the F1Fo ATPase, the ADP/ATP carrier, mitochondrial morphology components, or the i-AAA protease, Yme1p). Interestingly, other import mutants in our strain background are not petite-negative. We report that Tim54p is not involved in maintenance of mitochondrial DNA or mitochondrial morphology. Rather, Tim54p mediates assembly of an active Yme1p complex, after Yme1p is imported via the TIM23 pathway. Defective Yme1p assembly is likely the major contributing factor for the petite-negativity in strains lacking functional Tim54p. Thus, Tim54p has two independent functions: scaffolding/stability for the TIM22 membrane complex and assembly of Yme1p into a proteolytically active complex. As such, Tim54p links protein import, assembly, and turnover pathways in the mitochondrion.


PubMed PMC2064651 Online version:10.1083/jcb.200706195


ATP-Dependent Proteases; Adenosine Triphosphatases/metabolism; DNA, Mitochondrial/metabolism; Gene Expression; Glucose/metabolism; Membrane Transport Proteins/metabolism; Microbial Viability; Mitochondria/metabolism; Mitochondrial Membrane Transport Proteins/deficiency; Mitochondrial Membrane Transport Proteins/metabolism; Mitochondrial Membranes/metabolism; Models, Biological; Mutation/genetics; Protein Binding; Protein Processing, Post-Translational; Protein Transport; Saccharomyces cerevisiae/cytology; Saccharomyces cerevisiae/metabolism; Saccharomyces cerevisiae Proteins/metabolism



Gene product Qualifier GO Term Evidence Code with/from Aspect Extension Notes Status



GO:0045039: protein insertion into mitochondrial inner membrane

ECO:0000315: mutant phenotype evidence used in manual assertion


Seeded From UniProt


See also


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