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Comment
. 2023 Nov 2;146(11):4408-4410.
doi: 10.1093/brain/awad353.

Unravelling the role of huntingtin: from neurodevelopment to neurodegeneration

Jordan L Schultz  1   2   3 , Mohit Neema  1 , Peg C Nopoulos  1   2   4
Affiliations
Comment

Unravelling the role of huntingtin: from neurodevelopment to neurodegeneration

Jordan L Schultz et al. Brain. .

Abstract

This scientific commentary refers to ‘Genetic topography and cortical cell loss in Huntington’s disease link development and neurodegeneration’ by Estevez-Fraga et al. (https://doi.org/10.1093/brain/awad275).

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Conflict of interest statement

The authors report no competing interests.

Figures

Figure 1
Figure 1
Neurodevelopment and neurodegeneration in Huntington’s disease. Control subjects (green line) show normal brain development that peaks around the age of 30 with slow volumetric decline associated with normal ageing. Subjects with Huntington’s disease (purple line) experience brain hypertrophy earlier in life that may be associated with cognitive advantages. However, this advantageous neurodevelopment produces vulnerabilities leading to accelerated ageing processes and neurodegeneration.
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Comment on

References

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    1. Estevez-Fraga C, Altmann A, Parker CS, et al. Genetic topography and cortical cell loss in Huntington’s disease link development and neurodegeneration. Brain. 2023;146:4532-4546. - PMC - PubMed
    1. Arteaga-Bracho EE, Gulinello M, Winchester ML, et al. Postnatal and adult consequences of loss of huntingtin during development: Implications for Huntington’s disease. Neurobiol Dis. 2016;96:144–155. - PMC - PubMed
    1. Jacobsen JC, Gregory GC, Woda JM, et al. HD CAG-correlated gene expression changes support a simple dominant gain of function. Hum Mol Genet. 2011;20:2846–2860. - PMC - PubMed
    1. Lee J-M, Ramos EM, Lee J-H, et al. CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion. Neurology. 2012;78:690–695. - PMC - PubMed

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