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Novel interaction of the co-chaperone DNAJC12 with phenylalanine hydroxylase variants

Researchers from the lab of Aurora Martinez describe a novel mechanism for the degradation of enzymes involved in phenylketonuria and other neurometabolic disorders.

We showed that mutant phenylalanine hydroxylase (PAH) exhibits increased ubiquitination compared with normal PAH. We also showed that the co-chaperone DNAJC12 interacts with monoubiquitin-tagged PAH. The results indicate a role of DNAJC12 in the processing of misfolded ubiquitinated PAH.

Our image was featured on the cover of the April 2019 issue of Human Mutation.