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Treatment of PIRA remains an unmet need.

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Homeotic transformation score For Ubx/U1-70K heterozygous mutant with T2-T3 transformation, the transformation was scored as follows for 20 embryos: -: no changes, mild: VA muscles visible in T2 or T3, intermediate: VA muscles visible in T2 and T3, severe: VA muscles visible in T2 and T3 with LT muscles transformed into abdominal like phenotype

Kurzbach et al., 2015

Avoid freeze-thaw cycles: Once moved from freezer storage to refrigerator storage, maintain refrigeration

This severe depletion of GSH results in the synthesis of large amounts of -glutamylcysteine, which is increasingly converted to pyroglutamate when glycine is depleted, leading to metabolic acidosis