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Tracy Hampton, PhD

JAMA. 2007;298:505.

	Since this article does not have an abstract, we have provided the first 126 words of the full text and any section headings.

A team led by researchers at the University of Michigan in Ann Arbor has generated a new mouse model of neurodegeneration that carries a mutation also seen in some patients with the inherited nerve condition called Charcot-Marie-Tooth disorder (Chow CY et al. Nature. doi:10.1038/nature05876 [published online ahead of print June 17, 2007]).

The "pale tremor" mouse carries a mutation in a gene called Figure 4 encoding an enzyme that acts on certain phospholipids in the cell. The mice had abnormal pigmentation, early neurodegeneration, and large vacuoles in affected cells.

The scientists also identified pathogenic mutations of the human form of the gene, also called Figure 4, in 4 unrelated patients with autosomal recessive Charcot-Marie-Tooth disorder; they designated this form as CMT4J.

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