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The RPE and photoreceptors are highly metabolically active tissues as reflected by the above normal numbers of mitochondria present in them

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Deficiency in CSE promotes neurodegeneration in Huntingtons disease [18], whereas deficiency in ethylmalonic encephalopathy 1 (ETHE1 or persulfide dioxygenase, PDO), a H 2 S catabolizing enzyme, is a cause of ethylmalonic encephalopathy, which is characterized by abnormally high H 2 S levels in tissues and blood [19]

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For example, Monahan et al