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Spastic paraplegia proteins spastizin and spatacsin mediate autophagic lysosome reformation.
Together, these results link dysfunction of the autophagy/lysosomal biogenesis machinery to neurodegeneration.
FAM21 directs SNX27–retromer cargoes to the plasma membrane by preventing transport to the Golgi apparatus
SNX27 directly interacts with FAM21, which also binds retromer, within the Wiskott–Aldrich syndrome protein and SCAR homologue (WASH) complex, and the results demonstrate that the SNX27–retromer–WASH complex directs cargoes to the plasma membrane by blocking their transport to lysosomes and the Golgi.
A novel function of CEP135 as a platform protein of C-NAP1 for its centriolar localization.
Fibrous nanocellulose, crystalline nanocellulose, carbon nanotubes, and crocidolite asbestos elicit disparate immune responses upon pharyngeal aspiration in mice
Investigation of effects of four fibrous materials on pulmonary inflammation and immune responses found in the lungs, as well as the effects on spleen and peripheral blood immune cell subsets revealed alterations in immune response following pulmonary exposure should be taken into account when testing the applicability of new nanosized materials with fibrous morphology.
Protrudin binds atlastins and endoplasmic reticulum-shaping proteins and regulates network formation
- Jaerak Chang, Seongju Lee, C. Blackstone
- BiologyProceedings of the National Academy of Sciences
- 22 August 2013
It is shown that the SPG33 protein protrudin contains hydrophobic, intramembrane hairpin domains, interacts with tubular ER proteins, and functions in ER morphogenesis by regulating the sheet-to-tubule balance and possibly the density of tubule interconnections.
Essential role of Cenexin1, but not Odf2, in ciliogenesis
The results suggest that Cenexin1 plays a critical role in ciliogenesis through its C-terminal extension that confers a unique ability to mediate primary cilia assembly.
Spastin-Interacting Protein NA14/SSNA1 Functions in Cytokinesis and Axon Development
It is postulate that NA14 may act as an adaptor protein regulating spastin localization to centrosomes, temporally and spatially regulating the microtubule-severing activity of spASTin that is particularly critical during the cell cycle and neuronal development.
Lysosomal abnormalities in hereditary spastic paraplegia types SPG15 and SPG11
- B. Renvoisé, Jaerak Chang, T. Pierson
- Medicine, PsychologyAnnals of clinical and translational neurology
- 20 May 2014
SPG15 and SPG11 are clinically similar, autosomal recessive disorders characterized by progressive spastic paraplegia along with thin corpus callosum, white matter abnormalities, cognitive impairment, and ophthalmologic abnormalities.
Impaired mitochondrial dynamics underlie axonal defects in hereditary spastic paraplegias
Mechanisms by which long corticospinal axons degenerate in hereditary spastic paraplegia (HSP) are largely unknown. Here, we have generated induced pluripotent stem cells (iPSCs) from patients with…
Loss of AP-5 results in accumulation of aberrant endolysosomes: defining a new type of lysosomal storage disease
Describing three independent fibroblast lines derived from skin biopsies of patients harbouring nonsense mutations in AP5Z1 and presenting with spastic paraplegia accompanied by neuropathy, parkinsonism and/or cognitive impairment reveals an emerging picture of the role of AP-5 in endosomal and lysosomal homeostasis and expands the understanding of recessive HSPs.