Rescuing the disorganised brain

When did you last take for granted your ability to enjoy a cup of coffee without spilling it everywhere?

Ataxia, or loss of controlled movement, occurs when the electrical circuits in a part of your brain called the cerebellum start to get disorganised and go wrong.

The ataxias are a family of progressive and poorly treated movement disorders often accompanied by defective cerebellar function. The disorder can affect anyone, young or old, has a variety of causes, usually gets worse and is rarely reversible.

The search for better treatments requires improved understanding of how changes to the highly organised circuitry of the cerebellum contribute to the development of ataxia.

Using a unique mouse model, our research aims to better understand how the electrical connections within the cerebellum start to go wrong in ataxia.

We know that in our model the connections to the important brain cells, or neurones, in the cerebellum, called the Purkinje neurones are disorganised and we want to understand why!

The normally highly ordered structural architecture of the Purkinje brain cell is shown below and next to it is the same type of cell from the ataxia model.

If you think of the neurone as a tree, the branches provide the opportunity for the connections that signal important information. Look closely and you will see the disordered branches in the brain cell from the ataxic mice.

When we do something as apparently simple as picking up our mug of coffee, the branches of these cells make precisely timed connections that allow us to make the fine, smoothly controlled movement that we often take so for granted. In patients with ataxia this fine control is lost.

Using our model and state of the art imaging and electrical recordings we aim to watch and listen to the signalling events of the disorganised Purkinje neurones.

In this way we hope to understand just how they malfunction and more importantly to define and design new ways to rescue the disorganised brain cells and the important connections that they make.

- Dr Ruth Empson, Senior Lecturer, Department of Physiology

We thank the Neurological Foundation of New Zealand for generous funding to support this research.

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