Figure 7.

Structural Model of the Human mt-SSU Shows the Positions of Affected Proteins and Residues

(A) The human 28S mt-SSU (PDB: 3J9M⁷) was drawn in cartoon representation with PyMol version 1.7.4.0. The subunits known to be implicated in disease are shown with color-matched labels: magenta, MRPS2; raspberry, MRPS7; pale yellow, MRPS23; and shades of green, MPRS16, MRPS22, and MRPS34. Subunits interacting with MRPS2 are shown in shades of yellow, and constituents of the ∼300 kDa subassembly are shown in shades of blue and green. Other subunits are shown in gray, and 12S RNA is shown in light pink.

(B) Partial model of (A) shows only the 12S RNA (gray), MRPS2 (magenta), and surrounding proteins MRPS18C, MRPS21, and MRPS28 (shades of yellow). The positions of the residues of MRPS2 altered in S1 (Arg110 and Asp114) and in S3 (Arg138) are shown in green and cyan, respectively, as stick models.