Retinal degeneration in the mutant mouse: Accumulation of spherules in the interphotoreceptor space
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Facile purification of active recombinant mouse cytosolic carboxypeptidase 6 from Escherichia coli
2022, Protein Expression and PurificationCitation Excerpt :Polyglutamylation homeostasis is essential for multiple biological functions. Disrupted polyglutamylation due to loss of CCPs or TTLLs causes a broad spectrum of disorders in animals and humans, such as neurodegeneration, male infertility, retinal degeneration, and impaired immune function [5,10–12]. Recent studies using genetically manipulated models suggested that correcting the polyglutamylation balance by depleting the counteracting CCP or TTLL in respective TTLL and CCP mutants could attenuate some of these anomalies, and therefore pointing to potential therapeutic opportunities [13–15].
Phenotypic characterization of P23H and S334ter rhodopsin transgenic rat models of inherited retinal degeneration
2018, Experimental Eye ResearchCitation Excerpt :It is highly probable that the vesicles seen in the same region of the retina of other mutants also contain rhodopsin. In all cases, the origin of the vesicles appears to be from budding or blebbing from the ISs (Acland and Aguirre, 1987; Hagstrom et al., 1999; Kondo et al., 2009), or in some cases, protrusions from the ISs (Blanks et al., 1982; Blanks and Spee, 1992). Most of the vesicles are ultimately extracellular, as confirmed by scanning electron microscopy (Blanks and Spee, 1992; Li et al., 1996).
The Purkinje cell degeneration (pcd) mouse: An unexpected molecular link between neuronal degeneration and regeneration
2007, Brain ResearchCitation Excerpt :pcd mice lose about 50% of their retinal photoreceptor cells between 3 and 5 weeks of age, and thereafter slowly lose the remainder over a period of 1 year (Blanks and Spee, 1992). Transmission electron microscopy revealed ‘bead-like’ vesicles in the extracellular matrix surrounding the degenerating inner segment region of the photoreceptors at post-natal day 25 (Blanks and Spee, 1992). The membrane-bound vesicles varied in size from 125 to 370 nm.
Genetic animal models for retinal degeneration
2002, Survey of OphthalmologyExtracellular vesicles highlight many cases of photoreceptor degeneration
2023, Frontiers in Molecular Neuroscience