title: Altered palmitoylation and neuropathological deficits in mice lacking HIP14.
abstract: 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.
authors.affiliation: Centre for Molecular Medicine and Therapeutics and Child and Family Research Institute, Vancouver, BC, Canada.
authors.forename: Roshni R
authors.initials: RR
authors.lastname: Singaraja
authors.forename: Kun
authors.initials: K
authors.lastname: Huang
authors.forename: Shaun S
authors.initials: SS
authors.lastname: Sanders
authors.forename: Austen J
authors.initials: AJ
authors.lastname: Milnerwood
authors.forename: Rochelle
authors.initials: R
authors.lastname: Hines
authors.forename: Jason P
authors.initials: JP
authors.lastname: Lerch
authors.forename: Sonia
authors.initials: S
authors.lastname: Franciosi
authors.forename: Renaldo C
authors.initials: RC
authors.lastname: Drisdel
authors.forename: Kuljeet
authors.initials: K
authors.lastname: Vaid
authors.forename: Fiona B
authors.initials: FB
authors.lastname: Young
authors.forename: Crystal
authors.initials: C
authors.lastname: Doty
authors.forename: Junmei
authors.initials: J
authors.lastname: Wan
authors.forename: Nagat
authors.initials: N
authors.lastname: Bissada
authors.forename: R Mark
authors.initials: RM
authors.lastname: Henkelman
authors.forename: William N
authors.initials: WN
authors.lastname: Green
authors.forename: Nicholas G
authors.initials: NG
authors.lastname: Davis
authors.forename: Lynn A
authors.initials: LA
authors.lastname: Raymond
authors.forename: Michael R
authors.initials: MR
authors.lastname: Hayden
mesh_terms: D000217:Acyltransferases / Q000172:deficiency* / Q000235:genetics / Q000378:metabolism; D000818:Animals; D016923:Cell Death / Q000235:genetics; D003342:Corpus Striatum / Q000378:metabolism / Q000473:pathology; D004195:Disease Models, Animal; D051937:Dopamine and cAMP-Regulated Phosphoprotein 32 / Q000378:metabolism; D004745:Enkephalins / Q000378:metabolism; D006816:Huntington Disease / Q000209:etiology* / Q000235:genetics / Q000473:pathology; D054878:Lipoylation / Q000235:genetics*; D051379:Mice; D018345:Mice, Knockout; D009043:Motor Activity / Q000235:genetics; D050505:Mutant Proteins / Q000378:metabolism; D009419:Nerve Tissue Proteins / Q000172:deficiency* / Q000235:genetics / Q000378:metabolism; D009474:Neurons / Q000378:metabolism / Q000473:pathology; D050486:Serotonin Plasma Membrane Transport Proteins / Q000235:genetics / Q000378:metabolism; D013569:Synapses / Q000378:metabolism
chemical_list: D051937:Dopamine and cAMP-Regulated Phosphoprotein 32; D004745:Enkephalins; D050505:Mutant Proteins; D009419:Nerve Tissue Proteins; C497929:Ppp1r1b protein, mouse; D050486:Serotonin Plasma Membrane Transport Proteins; C493231:Slc6a4 protein, mouse; D000217:Acyltransferases; C569387:Zdhhc17 protein, mouse