StAR-related transfer domain

[1][4] The crystal structure of START domain of human MLN64 shows an alpha/beta fold built around a U-shaped incomplete beta-barrel.

Most importantly, the interior of the protein encompasses a 26 × 12 × 11-Angstrom hydrophobic tunnel that is apparently large enough to bind a single cholesterol molecule.

[5] The START domain structure revealed an unexpected similarity to that of the birch pollen allergen Bet v 1 and to bacterial polyketide cyclases/aromatases.

Mutations in STAR D9 (KIF16A) have been associated with a syndrome that includes severe ID, characteristic features, epilepsy, acquired microcephaly, and blindness.

In the reported case, several mitotic defects including multipolar spindle formation, fragmentation of pericentriolar materials, and centrosome amplification were found.