Abstract
BackgroundMutations in spastin are the most common cause of hereditary spastin paraplegia, a neurodegenerative disease. In this study, the role of spastin was examined in Drosophila photoreceptor development.Methodology/Principal FindingsThe spastin mutation in developing pupal eyes causes a mild mislocalization of the apical membrane domain at the distal section, but the apical domain was dramatically reduced at the proximal section of the developing pupal eye. Since the rhabdomeres in developing pupal eyes grow from distal to proximal, this phenotype strongly suggests that spastin is required for apical domain maintenance during rhabdomere elongation. This role of spastin in apical domain modulation was further supported by spastin's gain-of-function phenotype. Spastin overexpression in photoreceptors caused the expansion of the apical membrane domain from apical to basolateral in the developing photoreceptor. Although the localizations of the apical domain and adherens junctions (AJs) were severely expanded, there were no defects in cell polarity.Conclusions/SignificanceThese results strongly suggest that spastin is essential for apical domain biogenesis during rhabdomere elongation in Drosophila photoreceptor morphogenesis.
Highlights
Hereditary spastic paraplegia (HSP) comprises a heterogeneous group of neurological disorders [1]
Conclusions/Significance: These results strongly suggest that spastin is essential for apical domain biogenesis during rhabdomere elongation in Drosophila photoreceptor morphogenesis
We precisely identified the exact location of the stable microtubules in developing pupal eyes using the several markers of apical domain, adherens junctions (AJs) and basolateral junction
Summary
Hereditary spastic paraplegia (HSP) comprises a heterogeneous group of neurological disorders [1]. The autosomal dominant form of hereditary spastic paraplegia (AD-HSP) is a neurodegenerative disorder. It was suggested that the strong neurodegenerative defects observed in patients are caused by a primary defect of spastin in neurons [3]. Functions of Drosophila spastin were analyzed in developing neuromuscular junctions using genetic mutational studies. Together, these data provide the first in vivo evidence that Spastin regulates microtubule stability and show that this function of Spastin strongly modulates both synaptic architecture and neurotransmission strength in neuromuscular junctions [9,10]. Mutations in spastin are the most common cause of hereditary spastin paraplegia, a neurodegenerative disease. The role of spastin was examined in Drosophila photoreceptor development
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
