Fig. 6

Putative structure of DNAJB6b J and G/F domains obtained through homology modelling using the structure of TtDNAJ2 (pdbcode: 4 J80) as template. a Alignment of DNAJB6b and TtDNAJ2 highlighting the J and G/F domains. Identical residues are shown in bold, residues found mutated in patients are highlighted in red. b Ribbon representation of the homology model of DNAJB6. c A detail of the interface between J and G/F domains. The J domain is represented in surface and colored according to its electrostatic properties (blue for positive and red for negative charge, respectively) while the G/F domain is shown in cyan ribbon. Phenylalanine residues found at the interface are shown in sticks. d Detailed view of the interactions played by Phe100. Its corresponding valine residue found in patients is shown in magenta. The J domain is represented in blue while the G/F domain is represented in cyan. Residues close to Phe100 are also shown in sticks; Phe100 is predicted to establish interactions with both J domain residues (Val55, Lys61 and Ile64) and G/F domain residues (His82 and Val99). e Detailed view of the Phe93Leu mutation; Phe93 is predicted to establish a cation-π interation with J domain residue Lys47 and hydrophobic interactions with G/F domain Pro96. Pro96 faces the J domain helix III and its mutation to arginine is linked to distal-onset phenotype. f Detailed view of the Phe91Leu mutation; interacting residues are also shown. g Detailed view of the Phe89Ile mutation. Phe89 does not directly contact the J domain; rather it protrudes inside the G/F domain spiral where it is predicted to interact with Phe104. Color codes in (e, f and g) panels are the same as in (g)