A huntingtin–HAP1–PCM1 pathway in ciliogenesis

Abstract

Evaluation of: Keryer G, Pineda JR, Liot G et al. Ciliogenesis is regulated by a huntingtin–HAP1–PCM1 pathway and is altered in Huntington disease. J. Clin. Invest. 121(11), 4372–4382 (2011).

Huntington’s disease (HD) is caused by expansion of a polyglutamine repeat in the N-terminal region of huntingtin (htt), a large protein that has been found to interact with a variety of proteins. It remains to be determined how the interactions of htt with other proteins are involved in the pathogenesis of HD. A recent publication by Keryer et al. demonstrates that htt regulates ciliogenesis by interacting with PCM1 through HAP1. This recent study shows that htt and HAP1 are essential for protein trafficking to the centrosome, as well as normal ciliogenesis, and that mutant htt causes abnormal ciliogenesis, providing a novel insight into the pathogenesis of HD.

Keywords::

• centrosome
• ciliogenesis
• HAP1
• huntingtin
• Huntington disease

Financial & competing interests disclosure

The work performed in the authors’ laboratory was supported by grants from the NIH (AG019206 and NS041669 to X-J Li and AG031153 and NS045016 to S Li). The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.