The precursor of a mitochondrial matrix protein is found in a mutation in the tom22 signal receptor.
Tom22 and Tom20 function as N-terminal matrix targeting sequences' outer mitochondrial membrane receptor proteins. The buildup of mitochondrial matrix-targeted proteins in the cytosol due to a malfunctioning Tom22 receptor protein may be followed by cytosolic turnover of these proteins.
The N-terminal sequence, which directs the protein to mitochondria and is proteolytically processed upon import3, is produced in approximately 70% of mitochondrial precursor proteins3. To get to the matrix, they must pass via the TIM23 and TOM complexes on the inner and outer membranes, respectively.
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