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The process of lyophilization removes water, which is a key medium for many chemical degradation reactions

Conversely, the EAM mice displayed fragmented and sparse myofibrillar architecture, with rounded mitochondria, loss of inner membrane integrity, fragmented cristae, and swollen matrix

Treatment consists of replacement of kidney tubular losses, symptomatic management of systemic complications, and specific therapy directed at the basic defect, i.e., lysosomal cystine accumulation

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