Focal distortion of the nuclear envelope by huntingtin aggregates revealed by lamin immunostaining
2008

Huntingtin Aggregates Distort the Nuclear Envelope

publication Evidence: moderate

Author Information

Author(s): Chapple J. Paul, Bros-Facer Virginie, Butler Rachel, Gallo Jean-Marc

Primary Institution: MRC Centre for Neurodegeneration Research, King’s College London

Hypothesis

The study investigates how huntingtin aggregates affect the nuclear envelope in cells.

Conclusion

Huntingtin aggregates cause a focal distortion of the nuclear envelope in transfected cells.

Supporting Evidence

  • Huntingtin aggregates in a juxtanuclear position were associated with a clear focal distortion in the nuclear envelope.
  • Lamin B1 distribution was not altered by aggregates of polyglutamine-expanded ataxin-1.
  • Immunocytochemistry for lamin B1 can be used to monitor the state of the nuclear envelope in experimental studies.

Takeaway

When certain proteins related to Huntington's disease clump together, they can push against the nuclear envelope, changing its shape.

Methodology

The study used immunostaining for lamin B1 and laser confocal microscopy to analyze the nuclear envelope in transfected CHO cells.

Digital Object Identifier (DOI)

10.1016/j.neulet.2008.09.075

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