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Blachier J, Cleret A,Guerin N,Gil C, Fanjat JM,Tavernier F,Vidault L,Gallix F, Rama N, Rossignol RPD, Andrivon A

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cysteine is the rate-limiting amino acid for glutathione synthesis, a decrease of cysteine will result in the low glutathione levels seen in individuals with impairment of this pathway

Loss of CCP1 or CCP5 results in pathological hyperglutamylation that precedes and drives progressive structural disorganization of the outer segment, characterized by destabilization of the distal axoneme, loss of the bulge region required for disc formation, and severe impairment of IFT