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Article

Functional conservation of human Spastin in a Drosophila model of autosomal dominant-hereditary spastic paraplegia.

Human molecular genetics - 15 May 2010

Du Fang, Ozdowski Emily F, Kotowski Ingrid K, Marchuk Douglas A, Sherwood Nina Tang

Abstract excerpt

Mutations in spastin are the most frequent cause of the neurodegenerative disease autosomal dominant-hereditary spastic paraplegia (AD-HSP). Drosophila melanogaster lacking spastin exhibit striking behavioral similarities to human patients suffering from AD-HSP, suggesting conservation of Spastin function between the species. Consistent with this, we show that exogenous expression of wild-type Drosophila or human...

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Adenosine Triphosphatases
Animals, Genetically Modified
Genes, Dominant
Protein Transport
Spastic Paraplegia, Hereditary

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Subcellular Fractions

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PMID: 20154342
DOI: 10.1093/hmg/ddq064
PMCID: PMC2860889

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