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Molecular mechanism of the ichthyosis pathology of ChanarinDorfman syndrome: stimulation of PNPLA1-catalyzed - O -acylceramide production by ABHD5

With regard to mitochondrial autophagy, the absence of Park2an E3 ubiquitin ligase that plays a critical role in this processleads to mild cardiac abnormalities and persistent defects in mitochondrial metabolic function