YME1L1 is the human ortholog of yeast mitochondrial AAA metalloprotease, Yme1p. It is localized in the mitochondria and can functionally complement a yme1 disruptant yeast strain. It is proposed that this gene plays a role in mitochondrial protein metabolism and could be involved in mitochondrial pathologies. Two transcript variants encoding different isoforms have been found for this gene.
YME1L1 shares 50% sequence identity with yeast Yme1, 40% identity with yeast Rca1, and 39% identity with yeast Afg3, with highest homology in the central regions of the proteins. Northern blot analysis revealed transcripts of about 2.6 and 4.4 kb in all tissues examined, with greatest abundance in adult heart, skeletal muscle, and pancreas.
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