Mitochondrial inheritance · USMLE Step 1

Which of the following statements about nuclear-encoded mitochondrial proteins and their inheritance is correct?

  1. They follow maternal inheritance, just like mtDNA does
  2. They follow Mendelian, autosomal or X-linked, inheritance
  3. They are never implicated in mitochondrial disease
  4. They are encoded within the mtDNA genome itself
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Correct answer: They follow Mendelian, autosomal or X-linked, inheritance

Most mitochondrial proteins are encoded by nuclear genes and are inherited as autosomal (or occasionally X-linked) traits according to Mendelian genetics — unlike mtDNA-encoded proteins.

Difficulty: Medium Question 16 of 19

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