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Results 1 - 2 of 2 for Mitochondrial bifunctional protein deficiency
  1. ... complex in which it plays a part. This deficiency impairs the production of mitochondrial tRNAs. Without enough tRNAs, the mitochondrial synthesis of proteins involved in cellular energy production is reduced. A ...
  2. ... an essential step to generating tRNA molecules. Normal mitochondrial protein production, which requires tRNAs, is essential for the ... tRNA molecules. This reduction in functional tRNAs decreases mitochondrial protein synthesis and the production of energy in the ...