| Rare Neurological Diseases  

Huntingtin Aggregates in the Olfactory Bulb in Huntington’s Disease

Highet B, Dieriks BV, Murray HC, Faull RLM and Curtis MA (2020)

Huntingtin Aggregates in the Olfactory Bulb in Huntington’s Disease. Front. Aging Neurosci. 12:261. doi: 10.3389/fnagi.2020.00261


Non-motor signs and symptoms are known to often impair quality of life and function more than chorea in Huntington’s disease. Among those has been the intriguing observation of olfactory deficits, which may occur quite early in the course of the disease. Several hypotheses had been formulated to explain this disturbance, including cognitive impairment such as odor memory, identification and quality discrimination, but the idea of a more distal deficit was controversially debated. In this study the authors nicely describe the presence of typical aggregates containing huntingtin in the olfactory bulb, confirming its involvement in olfactory deficits. It will be very interesting to further explore the relationship between morphological findings and functional olfactory assessment over different stages of the disease. These findings provide a further example of similarities in pathogenetic mechanisms underlying neurodegenerative disorders. New studies in Parkinson’s disease examine the usefulness of nasal tissue assessment. The perspective to explore this in Huntington’s disease is quite exciting.



Bassil, F., Brown, H. J., Pattabhiraman, S., Iwasyk, J. E., Maghames, C. M., Meymand, E. S., et al. (2020). Amyloid-Beta (Aβ) plaques promote seeding and spreading of alpha-synuclein and Tau in a mouse model of lewy body disorders with Aβ pathology. Neuron 105, 260–275.e6. doi: 10.1016/j.neuron.2019.10.010

Blum, D., Herrera, F., Francelle, L., Mendes, T., Basquin, M., Obriot, H., et al. (2015). Mutant huntingtin alters Tau phosphorylation and subcellular distribution. Hum. Mol. Genet. 24, 76–85. doi: 10.1093/hmg/ddu421

Braak, H., Del Tredici, K., Rüb, U., de Vos, R. A. I., Jansen Steur, E. N. H., and Braak, E. (2003). Staging of brain pathology related to sporadic Parkinson’s disease. Neurobiol. Aging 24, 197–211. doi: 10.1016/S0197-4580(02)00065-9