Genomes and Genes
Gene Symbol: spastin
Alias: CG5977, D-spastin, Dmel\CG5977, Dspastin, Spas, Spastin, CG5977 gene product from transcript CG5977-RD, CG5977-PA, CG5977-PB, CG5977-PC, CG5977-PD, CG5977-PE, CG5977-PF, D-spastin, Dm-Spastin, dspastin, spas-PA, spas-PB, spas-PC, spas-PD, spas-PE, spas-PF, spasin
Species: fruit fly
- Identification of the Drosophila melanogaster homolog of the human spastin geneLars Kammermeier
Institute of Zoology, Biozentrum Pharmazentrum, University of Basel, Klingelbergstrasse 50, 4056 Basel, Switzerland
Dev Genes Evol 213:412-5. 2003The human SPG4 locus encodes the spastin gene, which is responsible for the most prevalent form of autosomal dominant hereditary spastic paraplegia (AD-HSP), a neurodegenerative disorder...
- Katanin p60-like1 promotes microtubule growth and terminal dendrite stability in the larval class IV sensory neurons of DrosophilaAndrea Stewart
Department of Biology, Duke University, Durham, North Carolina 27708, USA
J Neurosci 32:11631-42. 2012..Although loss of the related microtubule-severing protein Spastin also reduces the class IV dendrite arbor, microtubule polymerization within dendrites is unaffected...
- Drosophila spastin regulates synaptic microtubule networks and is required for normal motor functionNina Tang Sherwood
Broad Center, Division of Biology, California Institute of Technology Pasadena, California, USA
PLoS Biol 2:e429. 2004..form of human autosomal dominant hereditary spastic paraplegia (AD-HSP) is caused by mutations in the SPG4 (spastin) gene, which encodes an AAA ATPase closely related in sequence to the microtubule-severing protein Katanin...
- Loss of Drosophila melanogaster p21-activated kinase 3 suppresses defects in synapse structure and function caused by spastin mutationsEmily F Ozdowski
Department of Biology Institute for Genome Sciences and Policy, Duke University, Durham, North Carolina 27710, USA
Genetics 189:123-35. 2011..The AAA ATPase Spastin severs microtubules along their lengths and is thought to regulate the balance between long, stable filaments and ..
- The Drosophila homologue of the hereditary spastic paraplegia protein, spastin, severs and disassembles microtubulesAntonina Roll-Mecak
The Howard Hughes Medical Institute and the Department of Cellular and Molecular Pharmacology, University of California, San Francisco, California 94143, USA
Curr Biol 15:650-5. 2005..disorders characterized by lower-extremity spasticity and weakness, are most commonly caused by mutations in the spastin gene, which encodes a AAA+ ATPase related to the microtubule-severing protein katanin...
- Asymmetric localisation of cytokine mRNA is essential for JAK/STAT activation during cell invasivenessVeronique Van de Bor
Institute of Developmental Biology and Cancer UMR6543 CNRS, University of Nice Sophia Antipolis, Parc Valrose, 06108 Nice Cedex 2, France
Development 138:1383-93. 2011..These findings reveal a novel post-transcriptional regulatory mechanism of JAK/STAT signalling in the control of epithelial cell invasiveness...
- Disease-related phenotypes in a Drosophila model of hereditary spastic paraplegia are ameliorated by treatment with vinblastineGenny Orso
Dulbecco Telethon Institute and Department of Pharmacology, University of Padova, Padova, Italy
J Clin Invest 115:3026-34. 2005..Furthermore, its Drosophila homolog, Drosophila spastin (Dspastin), has been recently shown to regulate microtubule stability and synaptic function at the Drosophila larval ..
- Transiently reorganized microtubules are essential for zippering during dorsal closure in Drosophila melanogasterFerenc Jankovics
Cell Biology and Biophysics Unit, European Molecular Biology Laboratory, Meyerhofstrasse 1, 69117 Heidelberg, Germany
Dev Cell 11:375-85. 2006..We provide a clearly defined example where cells of a developing organism transiently reorganize their microtubules to fulfill a specialized morphogenetic task...
- Three microtubule severing enzymes contribute to the "Pacman-flux" machinery that moves chromosomesDong Zhang
Department of Physiology and Biophysics, Albert Einstein College of Medicine, Bronx, NY 10461, USA
J Cell Biol 177:231-42. 2007..in Drosophila melanogaster incorporates the activities of three different microtubule severing enzymes, Spastin, Fidgetin, and Katanin...
- A developmentally regulated two-step process generates a noncentrosomal microtubule network in Drosophila tracheal cellsVéronique Brodu
Institut Jacques Monod CNRS, 15 Rue H Brion, 75205 Paris Cedex 13, France
Dev Cell 18:790-801. 2010..MTOC components are first released from the centrosome by the activity of the MT-severing protein Spastin, and then anchored apically through the transmembrane protein Piopio...
- Structural basis of microtubule severing by the hereditary spastic paraplegia protein spastinAntonina Roll-Mecak
Howard Hughes Medical Institute and Department of Cellular and Molecular Pharmacology, University of California, San Francisco, 600 16th Street, San Francisco, California 94158, USA
Nature 451:363-7. 2008b>Spastin, the most common locus for mutations in hereditary spastic paraplegias, and katanin are related microtubule-severing AAA ATPases involved in constructing neuronal and non-centrosomal microtubule arrays and in segregating ..
- Clasp-mediated microtubule bundling regulates persistent motility and contact repulsion in Drosophila macrophages in vivoBrian Stramer
Randall Division of Cell and Molecular Biophysics, King s College London, London SE1 1UL, England, UK
J Cell Biol 189:681-9. 2010..align just before cell-cell repulsion, and we show that forcing depolymerization of microtubules by expression of Spastin leads to their defective polarity and failure to contact inhibit from one another...
- Functional conservation of human Spastin in a Drosophila model of autosomal dominant-hereditary spastic paraplegiaFang Du
Department of Biology and Institute for Genome Sciences and Policy, Duke University, Durham, NC 27708, USA
Hum Mol Genet 19:1883-96. 2010Mutations in spastin are the most frequent cause of the neurodegenerative disease autosomal dominant-hereditary spastic paraplegia (AD-HSP)...
- Mechanical control of global cell behaviour during dorsal closure in DrosophilaNicole Gorfinkiel
Department of Genetics, University of Cambridge, Cambridge CB2 3EH, UK
Development 136:1889-98. 2009..Our mutant analysis reveals the contribution of mechanical elements to the rate and pattern of DC...
- The hereditary spastic paraplegia gene, spastin, regulates microtubule stability to modulate synaptic structure and functionNick Trotta
Department of Biological Sciences, Vanderbilt Kennedy Center for Research on Human Development, Vanderbilt University, Nashville, TN 37235, USA
Curr Biol 14:1135-47. 2004..Over 20 genes have been linked to HSP in humans; however, mutations in one gene, spastin (SPG4), are the cause of >40% of all cases...
- Neuropathology: many paths lead to hereditary spastic paraplegiaRobert M Gould
Department of Anatomy and Cell Biology, University of Illinois at Chicago, Chicago, Illinois 60612, USA
Curr Biol 14:R903-4. 2004..Studies with animal models are providing new insights into the pathology of hereditary spastic paraplegia, particularly how mutations in multiple, converging pathways can lead to this family of neuropathies...
- Conserved pharmacological rescue of hereditary spastic paraplegia-related phenotypes across model organismsCarl Julien
CRCHUM and Department of Neuroscience and
Hum Mol Genet 25:1088-99. 2016..We found that locomotor defects found in all of our spastin models could be partially rescued by phenazine, methylene blue, N-acetyl-cysteine, guanabenz and salubrinal...
- The Krüppel-Like Factor Dar1 Determines Multipolar Neuron MorphologyXin Wang
Life Sciences Institute, Graduate Program in Molecular, Cellular, and Developmental Biology
J Neurosci 35:14251-9. 2015..Dar1 likely functions as a terminal selector gene for the basic layout of neuron morphology by regulating both dendrite extension and the dendrite-nucleus coupling...
- Drosophila KASH-domain protein Klarsicht regulates microtubule stability and integrin receptor localization during collective cell migrationM M Myat
Department of Biology, Medgar Evers College CUNY, 1638 Bedford Avenue, Brooklyn, NY 11225, USA Electronic address
Dev Biol 407:103-14. 2015..cell rearrangement and integrin localization observed in klar mutants could be mimicked by overexpressing Spastin (Spas), a MT severing protein, in otherwise wild-type salivary glands...
- Spastin binds to lipid droplets and affects lipid metabolismChrisovalantis Papadopoulos
Institute for Genetics, University of Cologne, Cologne, Germany Cologne Excellence Cluster on Cellular Stress Responses in Aging Associated Diseases CECAD, Cologne, Germany
PLoS Genet 11:e1005149. 2015..Consistent with these findings, ubiquitous overexpression of Dspastin in Drosophila led to bigger and less numerous LDs in the fat bodies and increased triacylglycerol levels...
- BMP signaling and microtubule organization regulate synaptic strengthR W Ball
Department of Molecular and Cell Biology and the Helen Wills Neuroscience Institute, University of California, Berkeley, CA 94720, United States
Neuroscience 291:155-66. 2015..The spastin mutation, which disorganizes microtubules, disrupted the transmission gradient, supporting this interpretation...
- Microtubule-dependent apical restriction of recycling endosomes sustains adherens junctions during morphogenesis of the Drosophila tracheal systemPierre Marie Le Droguen
Institut Jacques Monod, CNRS and University Paris Diderot, 15 Rue H Brion, Paris 75205, Cedex 13, France
Development 142:363-74. 2015..Altogether, our results uncover an MT-Dynein-dependent apical restriction of recycling endosomes that controls adhesion by sustaining Par-3 and E-Cadherin levels at AJs during morphogenesis. ..
- Isoform-specific functions of Mud/NuMA mediate binucleation of Drosophila male accessory gland cellsKiichiro Taniguchi
Department of Life Science, Faculty of Science, Gakushuin University, Tokyo, 171 8588, Japan
BMC Dev Biol 14:46. 2014..We investigated them by characterizing the binucleation event during development of the Drosophila male accessory gland, in which all cells are binucleate...
- Regulatory mechanisms underlying the differential growth of dendrites and axonsXin Wang
Life Sciences Institute and Department of Cell and Developmental Biology, University of Michigan, Ann Arbor, MI, 48109, USA
Neurosci Bull 30:557-68. 2014..The knowledge of these underlying molecular mechanisms not only expands our understanding about how neural circuits are wired, but also provides insights that will aid in the rational design of therapies for neurological diseases...
- A dynamic microtubule cytoskeleton directs medial actomyosin function during tube formationAlexander J R Booth
MRC Laboratory of Molecular Biology, Cambridge Biomedical Campus, Francis Crick Avenue, Cambridge CB2 0QH, UK
Dev Cell 29:562-76. 2014..The medial actomyosin network interacts with the minus ends of acentrosomal microtubule bundles through the cytolinker protein Shot, and disruption of Shot also impairs apical constriction...
- Cold temperature improves mobility and survival in Drosophila models of autosomal-dominant hereditary spastic paraplegia (AD-HSP)Sally L Baxter
Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA
Dis Model Mech 7:1005-12. 2014..Over half of AD-HSP cases arise from loss-of-function mutations in spastin, which encodes a microtubule-severing AAA ATPase...
- Microtubule-severing protein Katanin regulates neuromuscular junction development and dendritic elaboration in DrosophilaChuan Xi Mao
College of Life Science, Hubei University, Wuhan, Hubei 430062, China
Development 141:1064-74. 2014..Taken together, our results demonstrate for the first time that Katanin 60 is required for the normal development of neuromuscular synapses and dendrites. ..
- Dynamic microtubules produce an asymmetric E-cadherin-Bazooka complex to maintain segment boundariesNatalia A Bulgakova
Wellcome Trust Cancer Research UK Gurdon Institute, University of Cambridge, Cambridge CB2 1QN, England, UK
J Cell Biol 201:887-901. 2013....
- Microtubules and Lis-1/NudE/dynein regulate invasive cell-on-cell migration in DrosophilaNachen Yang
Institute of Molecular and Cell Biology, Singapore, Singapore
PLoS ONE 7:e40632. 2012..Spatial regulation of cell-cell adhesion may be a common theme, consistent with observing both cell autonomous and non-autonomous requirements in both systems...
- The centrosome in human genetic diseaseJose L Badano
McKusick Nathans Institute of Genetic Medicine, John Hopkins University, 533 Broadway Research Building, 733 N Broadway, Baltimore, Maryland 21205, USA
Nat Rev Genet 6:194-205. 2005..Here, we review the mechanistic relationship between human disease phenotypes and the function of the centrosome, and describe some of the newly-appreciated functions of this organelle in animal cells...
- JNK signalling influences intracellular trafficking during Drosophila morphogenesis through regulation of the novel target gene Rab30Chloe Thomas
Institute of Developmental Biology and Cancer, University of Nice, UMR CNRS, France
Dev Biol 331:250-60. 2009..We propose that JNK activity upregulates genes involved in intracellular transport in order to provide an increased level of trafficking activity in cells undergoing complex morphogenetic arrangements such as dorsal closure...
- Non-cell-autonomous control of denticle diversity in the Drosophila embryoStacie A Dilks
Department of Cell and Developmental Biology, University of Pennsylvania School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
Development 137:1395-404. 2010..We propose that stripe mediates its effect on hook orientation, in part, via upregulation of shot...
- A mathematical model for dorsal closureLuis Almeida
Laboratoire J A Dieudonne, Universite de Nice, UMR 6621 CNRS, Parc Valrose, F 06108 Nice Cedex 02, France
J Theor Biol 268:105-19. 2011..native setting and also test it further in embryos where the zipping force is perturbed through the expression of spastin (a microtubule severing protein)...
- Integrin adhesion drives the emergent polarization of active cytoskeletal stresses to pattern cell delaminationC Meghana
Department of Biological Sciences, Tata Institute of Fundamental Research, Mumbai 400005, India
Proc Natl Acad Sci U S A 108:9107-12. 2011..We suggest that differential contributions of adhesion, cytoskeletal, and external stresses must underlie differences in spatial pattern...
- Spastin's microtubule-binding properties and comparison to kataninThomas Eckert
Department of Physics E22 Biophysics, Technische Universitat Munchen, Garching, Germany
PLoS ONE 7:e50161. 2012b>Spastin and katanin are ring-shaped hexameric AAA ATPases that sever microtubules, and thus crucially depend on a physical interaction with microtubules...
- Normal spastin gene dosage is specifically required for axon regenerationMichelle C Stone
Department of Biochemistry and Molecular Biology, Penn State, University Park, PA 16802, USA
Cell Rep 2:1340-50. 2012..We found that axon regeneration is extremely sensitive to levels of the microtubule-severing protein spastin. Although microtubule behavior in uninjured neurons was not perturbed in animals heterozygous for a spastin null ..
- Axon injury and stress trigger a microtubule-based neuroprotective pathwayLi Chen
Department of Biochemistry and Molecular Biology, Penn State University, University Park, PA 16802, USA
Proc Natl Acad Sci U S A 109:11842-7. 2012..We conclude that axon injury or stress triggers a microtubule-based neuroprotective pathway that stabilizes neurons against degeneration...
- Differential regulation of dendritic and axonal development by the novel Krüppel-like factor Dar1Bing Ye
Life Sciences Institute and Department of Cell and Developmental Biology, University of Michigan, Ann Arbor, Michigan 48109, USA
J Neurosci 31:3309-19. 2011..that Dar1 promotes dendrite growth in part by suppressing the expression of the microtubule-severing protein Spastin. Our study thus uncovers a novel transcriptional program for microtubule regulation that preferentially controls ..
- The GTPase dMiro is required for axonal transport of mitochondria to Drosophila synapsesXiufang Guo
Arizona Research Laboratories, Division of Neurobiology, University of Arizona, Tucson, Arizona 85721, USA
Neuron 47:379-93. 2005..Together, our findings suggest that dMiro is required for controlling anterograde transport of mitochondria and their proper distribution within nerve terminals...
- Knot/Collier and cut control different aspects of dendrite cytoskeleton and synergize to define final arbor shapeShiho Jinushi-Nakao
RIKEN Brain Science Institute, Wako Shi, Saitama 351 0198, Japan
Neuron 56:963-78. 2007..Knot increases dendritic arbor outgrowth through promoting the expression of Spastin, a microtubule-severing protein disrupted in autosomal dominant hereditary spastic paraplegia (AD-HSP)...
- Roles of type I myosins in Drosophila handednessKiichiro Taniguchi
Department of Biological Science and Technology, Tokyo University of Science, Noda, Chiba, Japan
Fly (Austin) 1:287-90. 2007..We propose that the actin-based functions of type I Myosins play critical roles in generating LR asymmetry in invertebrates...
- Role of spastin in apical domain control along the rhabdomere elongation in Drosophila photoreceptorGeng Chen
Department of Biology, Baylor University, Waco, Texas, United States of America
PLoS ONE 5:e9480. 2010Mutations 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.
- Drosophila Tubulin-specific chaperone E functions at neuromuscular synapses and is required for microtubule network formationShan Jin
Institute of Genetics and Developmental Biology, Chinese Academy of Sciences, Beijing 100101, China
Development 136:1571-81. 2009..Genetic analyses revealed an antagonistic interaction between TBCE and the microtubule-severing protein Spastin. Moreover, treatment of muscles with the microtubule-depolymerizing drug nocodazole implicated TBCE as a tubulin ..
- In vivo coupling of cell elongation and lumen formation in a single cellLouis Gervais
Institut de Biologia Molecular de Barcelona CSIC, Baldiri Reixac 10 12, 08028 Barcelona, Spain
Curr Biol 20:359-66. 2010..Given the similarities in the formation of fine respiratory tubes and capillaries, we propose that an inward membrane growth model could account for lumen formation in both processes...
- Drosophila Atlastin regulates the stability of muscle microtubules and is required for synapse developmentMihye Lee
Department of Cell and Developmental Biology, Dental Research Institute, School of Dentistry, Seoul National University, Seoul 110 740, Republic of Korea
Dev Biol 330:250-62. 2009..We also provide evidence that Atl functions with the microtubule-severing protein Spastin to disassemble microtubules in muscles...