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. Author manuscript; available in PMC: 2011 May 10.
Published in final edited form as: J Inherit Metab Dis. 2010 Nov 20;34(1):225–231. doi: 10.1007/s10545-010-9237-9

Figure 2. Predicted CPOX Structures for the Wild-type and p.H327R Mutant Enzymes.

Figure 2

Panel A indicates the interacting region in the wild-type structure involved in the enzyme’s active site conversion of harderoporphyrinogen to protoporphyrinogen IX with the inter-atomic distances given between residues H327 and Y399. Panel B shows the modeled p.H327R substitution and close inter-atomic distances indicating that a clash would distort the region on helix α9 near residue R401, a mutation which previously was shown to result in harderoporphryin accumulation in vitro.