English Journal

The palmitoyl acyltransferase HIP14 shares a high proportion of interactors with huntingtin: implications for a role in the pathogenesis of Huntington's disease.

Butland SL1, Sanders SS1, Schmidt ME1, Riechers SP2, Lin DT1, Martin DD1, Vaid K1, Graham RK1, Singaraja RR1, Wanker EE3, Conibear E4, Hayden MR4.
Human molecular genetics.Hum Mol Genet.2014 Aug 1;23(15):4142-60. doi: 10.1093/hmg/ddu137. Epub 2014 Apr 4.
HIP14 is the most highly conserved of 23 human palmitoyl acyltransferases (PATs) that catalyze the post-translational addition of palmitate to proteins, including huntingtin (HTT). HIP14 is dysfunctional in the presence of mutant HTT (mHTT), the causative gene for Huntington disease (HD), and we hyp
PMID 24705354

Loss of function mutation in the palmitoyl-transferase HHAT leads to syndromic 46,XY disorder of sex development by impeding Hedgehog protein palmitoylation and signaling.

Callier P1, Calvel P2, Matevossian A3, Makrythanasis P2, Bernard P4, Kurosaka H5, Vannier A2, Thauvin-Robinet C6, Borel C2, Mazaud-Guittot S7, Rolland A7, Desdoits-Lethimonier C7, Guipponi M2, Zimmermann C2, Stévant I2, Kuhne F2, Conne B2, Santoni F2, Lambert S8, Huet F6, Mugneret F8, Jaruzelska J9, Faivre L6, Wilhelm D4, Jégou B10, Trainor PA11, Resh MD3, Antonarakis SE12, Nef S12.
PLoS genetics.PLoS Genet.2014 May 1;10(5):e1004340. doi: 10.1371/journal.pgen.1004340. eCollection 2014.
The Hedgehog (Hh) family of secreted proteins act as morphogens to control embryonic patterning and development in a variety of organ systems. Post-translational covalent attachment of cholesterol and palmitate to Hh proteins are critical for multimerization and long range signaling potency. However
PMID 24784881

Identification of binding sites in Huntingtin for the Huntingtin Interacting Proteins HIP14 and HIP14L.

Sanders SS1, Mui KK1, Sutton LM1, Hayden MR1.
PloS one.PLoS One.2014 Feb 28;9(2):e90669. doi: 10.1371/journal.pone.0090669. eCollection 2014.
Huntington disease is an adult onset neurodegenerative disease characterized by motor, cognitive, and psychiatric dysfunction, caused by a CAG expansion in the HTT gene. Huntingtin Interacting Protein 14 (HIP14) and Huntingtin Interacting Protein 14-like (HIP14L) are palmitoyl acyltransferases (PATs
PMID 24651384