YME1L1 consists of 716 amino acids and is highly similar to all mitochondrial AAA proteases and in particular to yeast Yme1p.

[7] YME1L1 is embedded in the inner mitochondrial membrane and is more abundant in tissues with a high content of mitochondria such as human adult heart, skeletal muscle, and pancreas RNA.

[7][6] YME1L1 is a member of the AAA family of ATPases and has an important role for the maintenance of mitochondrial morphology.

[12] It's also reported that YME1L1 controls the accumulation of respiratory chain subunits[13] and is required for apoptotic resistance, cristae morphogenesis, and cell proliferation.

[14] A homozygous mutation in the YME1L1 gene would cause infantile-onset mitochondriopathy, with severe intellectual disability, muscular impairments, and optic nerve atrophy.