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Mutations in the <jats:italic>SPG3A<\/jats:italic> gene, which encodes the large guanosine triphosphatase atlastin, are the second most common cause of autosomal dominant hereditary spastic paraplegia. In a large <jats:italic>SPG3A<\/jats:italic> screen of 70 hereditary spastic paraplegia subjects, a novel in\u2010frame deletion, p.del436N, was identified. Characterization of this deletion showed that it affects neither the guanosine triphosphatase activity of atlastin nor interactions between atlastin and spastin. Interestingly, immunoblot analysis of lymphoblasts from affected patients demonstrated a significant reduction in atlastin protein levels, supporting a loss\u2010of\u2010function disease mechanism. 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