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Iron deposits appear early in disease progression and manifest as round cytoplasmic inclusions in motor neurons, with minimal involvement of dorsal structures (352, 355)

By contrast, the k cat values of the ketosteroid isomerase activity of human, equine, and marmoset GST A3-3 are two orders of magnitude higher, ranging between 204 and 261 s 1 ( Our enzymological studies have established 5-PD and 5-AD as outstanding substrates for the equine and human GST A3-3 enzymes, and we predict that the corresponding 3-keto intermediates derived by oxidation of the 3-hydroxy groups of 17-hydroxypregnenolone and androstenediol will also be substrates, although they have not yet been tested