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A recent report (1) presents the long-awaited confirmation of paternal inheritance of mtDNA in humans (2). Surprisingly, paternal transmission of mtDNA (1) follows a bimodal pattern: About half of the offspring show fairly uniform paternal/maternal heteroplasmy levels, while the rest do not inherit paternal mtDNA at all. This pattern resembles the inheritance of a dominant nuclear gene. The authors explain this pattern as permissive inheritance resulting from a faulty “gatekeeper” gene (1). However, 3 groups (3⇓–5) instead suspect contamination with mtDNA nuclear pseudogenes (NUMTs), a notorious artifact (6).Based on our vast NUMT experience, we support the authors’ response (7), asserting that NUMT artifact is unlikely (further explanation is given in Supporting Notes [SN] sections SN1 and SN2, see ref. 8). However, we also demonstrate that the authors’ dominant gatekeeper explanation is incorrect, because spermatozoa are functionally diploid (SN3) … [↵][1]2To whom correspondence may be addressed. Email: p.j.i.ellis{at}kent.ac.uk or kkhrapko{at}gmail.com. [1]: #xref-corresp-1-1

Quasi-Mendelian paternal inheritance of mitochondrial DNA: A notorious artifact, or anticipated behavior?