Research ArticleCOCKAYNE SYNDROME

HDAC inhibition improves autophagic and lysosomal function to prevent loss of subcutaneous fat in a mouse model of Cockayne syndrome

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Science Translational Medicine  29 Aug 2018:
Vol. 10, Issue 456, eaam7510
DOI: 10.1126/scitranslmed.aam7510

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Autophagy and adipose

Hypersensitivity to sunlight and decreased subcutaneous fat are characteristics of Cockayne syndrome, a genetic disorder of premature aging. Majora and colleagues studied the role of autophagy underlying this skin phenotype using mice with a mutation in csb, a gene that causes Cockayne syndrome. They found an accumulation of autophagy-related proteins and lysosomal dysfunction in skin from csb mutant mice exposed to ultraviolet light. Treatment with a histone deacetylase inhibitor prevented loss of subcutaneous fat and rescued autophagic/lysosomal dysfunction. Further research is necessary to determine whether histone deacetylase inhibition can resolve other features of Cockayne syndrome, such as neurodegeneration.