The DOI matches its Crossref record; 3 of the 3 references it cites were found in a registry or an index.
Motor Deficit in a Drosophila Model of Mucolipidosis Type IV due to Defective Clearance of Apoptotic Cells
Open accessAbstract
Summary Disruption of the TRPML1 channel results in the neurodegenerative disorder mucolipidosis type IV (MLIV), a lysosomal storage disease with severe motor impairments. The mechanisms underlying MLIV are poorly understood and there is no treatment. Here, we report a Drosophila MLIV model, which recapitulates the key disease features, including abnormal intracellular accumulation of macromolecules, motor defects and neurodegeneration. The basis for the buildup of macromolecules was defective autophagy, which resulted in oxidative stress and impaired synaptic transmission. Late-apoptotic cells accumulated in trpml mutant brains suggesting diminished cell clearance. The accumulation of late apoptotic cells and motor deficits were suppressed by expression of trpml+ in neurons, glia or hematopoietic cells. We conclude that the neurodegeneration and motor defects result primarily from decreased clearance of apoptotic cells. Since hematopoietic cells in humans are involved in clearance of apoptotic cells, our results raise the possibility that bone marrow transplantation may limit the progression of MLIV.
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Cites
Fission and selective fusion govern mitochondrial segregation and elimination by autophagy
Twig, G., Elorza, A., Molina, A. J. A., Mohamed, H., Wikstrom, J. D., Walzer, G., Stiles, L., Haigh, S. E., Katz, S., Las, G., et al. (2008) · The EMBO Journal · doi:10.1038/sj.emboj.7601963 · Cited by 3,082 · cited 3×
“Mitochondria undergo cycles of fusion and fission, which sometimes gives rise touneven daughters, oneof which hasa disrupted transmembrane potential (DJ) and is targeted for degradation via autophagy (Twig et al., 2008).”
BackgroundInfluentialRavikumar, B., Vacher, C., Berger, Z., Davies, J. E., Luo, S., Oroz, L. G., Scaravilli, F., Easton, D. F., Duden, R., O'Kane, C. J., et al. (2004) · Nature Genetics · doi:10.1038/ng1362 · Cited by 2,347
St-Pierre, J., Drori, S., Uldry, M., Silvaggi, J. M., Rhee, J., Jäger, S., Handschin, C., Zheng, K., Lin, J., Yang, W., et al. (2006) · Cell · doi:10.1016/j.cell.2006.09.024 · Cited by 2,233
Cited by
Therapeutic potential of mood stabilizers lithium and valproic acid:Beyond bipolar disorder
Chiu, C.-T., Wang, Z., Hunsberger, J. G., & Chuang, D.-M. (2013) · Pharmacological Reviews · doi:10.1124/pr.111.005512 · Cited by 404
A TRP Channel in the Lysosome Regulates Large Particle Phagocytosis via Focal Exocytosis
Samie, M., Wang, X., Zhang, X., Goschka, A., Li, X., Cheng, X., Gregg, E., Azar, M., Zhuo, Y., Garrity, A. G., et al. (2013) · Developmental Cell · doi:10.1016/j.devcel.2013.08.003 · Cited by 317
Regulation of lysosomal ion homeostasis by channels and transporters
Xiong, J., & Zhu, M. X. (2016) · Science China Life Sciences · doi:10.1007/s11427-016-5090-x · Cited by 141
Lysosomal Degradation Is Required for Sustained Phagocytosis of Bacteria by Macrophages
Wong, C.-O., Gregory, S., Hu, H., Chao, Y., Sepúlveda, V. E., He, Y., Li-Kroeger, D., Goldman, W. E., Bellen, H. J., & Venkatachalam, K. (2017) · Cell Host & Microbe · doi:10.1016/j.chom.2017.05.002 · Cited by 114
Self-citationIdentification of the Penta-EF-hand Protein ALG-2 as a Ca2+-dependent Interactor of Mucolipin-1
Vergarajauregui, S., Martina, J. A., & Puertollano, R. (2009) · Journal of Biological Chemistry · doi:10.1074/jbc.m109.047241 · Cited by 85
Metadata
- Authors
- Venkatachalam, K., Long, A. A., Elsaesser, R., Nikolaeva, D., Broadie, K., & Montell, C.
- Venue
- Cell
- Publisher
- Elsevier BV
- Published
- 2008-11
- Volume
- 135
- Issue
- 5
- Pages
- 838-851
- ISSN
- 0092-8674 (ISSN-L)
- OpenAlex
- W2050340611
- Cited by
- 218
- References
- 3
- FWCI
- 10.19
- Type
- journal article
- Language
- en
- Open access
- bronze
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- The paper
- Venkatachalam, K., Long, A. A., Elsaesser, R., Nikolaeva, D., Broadie, K., & Montell, C. (2008). Motor Deficit in a Drosophila Model of Mucolipidosis Type IV due to Defective Clearance of Apoptotic Cells. Cell. https://doi.org/10.1016/j.cell.2008.09.041
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
