Altered palmitoylation and neuropathological deficits in mice lacking HIP14

Singaraja Roshni R; Huang Kun; Sanders Shaun S; Milnerwood Austen J; Hines Rochelle; Lerch Jason P; Franciosi Sonia; Drisdel Renaldo C; Vaid Kuljeet; Young Fiona B; Doty Crystal; Wan Junmei; Bissada Nagat; Henkelman R Mark; Green William N; Davis Nicholas G; Raymond Lynn A; Hayden Michael R<sup>*</sup>

doi:10.1093/hmg/ddr308

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Altered palmitoylation and neuropathological deficits in mice lacking HIP14

作者：Singaraja Roshni R; Huang Kun; Sanders Shaun S; Milnerwood Austen J; Hines Rochelle; Lerch Jason P; Franciosi Sonia; Drisdel Renaldo C; Vaid Kuljeet; Young Fiona B; Doty Crystal; Wan Junmei; Bissada Nagat; Henkelman R Mark; Green William N; Davis Nicholas G; Raymond Lynn A; Hayden Michael R^*

来源：Human Molecular Genetics, 2011, 20(20): 3899-3909.

DOI：10.1093/hmg/ddr308

摘要

Huntingtin interacting protein 14 (HIP14, ZDHHC17) is a huntingtin (HTT) interacting protein with palmitoyl transferase activity. In order to interrogate the function of Hip14, we generated mice with disruption in their Hip14 gene. Hip14-/- mice displayed behavioral, biochemical and neuropathological defects that are reminiscent of Huntington disease (HD). Palmitoylation of other HIP14 substrates, but not Htt, was reduced in the Hip14-/- mice. Hip14 is dysfunctional in the presence of mutant htt in the YAC128 mouse model of HD, suggesting that altered palmitoylation mediated by HIP14 may contribute to HD.