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Figure 2.
Figure 2. FAH structure, topology and HT1-associated
mutations. (a) A stereo ribbon diagram illustrating the FAH
subunit structure and position of point mutations causing
hereditary tyrosinemia type I is shown. The N-terminal domain is
located at the bottom of the figure. The mixed b-sandwich roll
structure is centrally located in the figure. Helices are
colored red; b strands are colored in shades of blue
corresponding to the b sheet they form; the positions of point
mutations are represented by green spheres; a calcium ion is
colored yellow; acetate carbon and oxygen atoms are respectively
colored orange and red (top of figure). (b) A topology diagram
of the novel FAH b-strand arrangement is shown. b Strands are
numbered in red according to their sequential occurrence in the
polypeptide chain; residue numbering is in black. Sheets A, B
and C are respectively colored in dark, light and medium shades
of blue, as in (a). a Helices are represented by red rectangles.
Figure 2, Figure 3 and Figure 4 and Figure 6b were generated
using MOLSCRIPT [40].
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