Inheritance is usually autosomal recessive, however, an autosomal dominant inheritance has also
been suggested. Mutations in the BCS1L gene on chromosome 2q33 or the UQCRB gene on chromosome 8 have been
demonstrated. The BCS1L gene are associated with tubulopathy, encephalopathy, and liver failure. GRACILE syndrome,
which belongs to the Finnish disease heritage, is also caused by mutation in the BCS1L gene, but displays a different
phenotype.