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1.
J Cereb Blood Flow Metab ; 41(12): 3213-3231, 2021 12.
Artículo en Inglés | MEDLINE | ID: mdl-34187232

RESUMEN

Autophagy is essential to cell function, as it enables the recycling of intracellular constituents during starvation and in addition functions as a quality control mechanism by eliminating spent organelles and proteins that could cause cellular damage if not properly removed. Recently, we reported on Wdfy3's role in mitophagy, a clinically relevant macroautophagic scaffold protein that is linked to intellectual disability, neurodevelopmental delay, and autism spectrum disorder. In this study, we confirm our previous report that Wdfy3 haploinsufficiency in mice results in decreased mitophagy with accumulation of mitochondria with altered morphology, but expanding on that observation, we also note decreased mitochondrial localization at synaptic terminals and decreased synaptic density, which may contribute to altered synaptic plasticity. These changes are accompanied by defective elimination of glycogen particles and a shift to increased glycogen synthesis over glycogenolysis and glycophagy. This imbalance leads to an age-dependent higher incidence of brain glycogen deposits with cerebellar hypoplasia. Our results support and further extend Wdfy3's role in modulating both brain bioenergetics and synaptic plasticity by including glycogen as a target of macroautophagic degradation.


Asunto(s)
Proteínas Adaptadoras Transductoras de Señales/metabolismo , Proteínas Relacionadas con la Autofagia/metabolismo , Encéfalo/metabolismo , Gluconeogénesis , Glucógeno/biosíntesis , Mitocondrias/metabolismo , Mitofagia , Plasticidad Neuronal , Proteínas Adaptadoras Transductoras de Señales/genética , Animales , Proteínas Relacionadas con la Autofagia/genética , Glucógeno/genética , Haploinsuficiencia , Ratones , Ratones Transgénicos , Mitocondrias/genética
2.
PLoS One ; 13(2): e0191323, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-29444089

RESUMEN

Proctophyllodes huitzilopochtlii Atyeo & Braasch 1966 (Acariformes: Astigmata: Proctophyllodidae), a feather mite, was found on feathers collected from five hummingbird species in California. This mite has not been previously documented on feathers from Anna's (Calypte anna [Lesson 1829]) or Black-chinned (Archilochus alexandri [Bourcier & Mulsant 1846]) Hummingbirds. A total of 753 hummingbirds were evaluated for the presence of mites by species (Allen's n = 112; Anna's n = 500; Black-chinned n = 122; Rufous n = 18; Calliope n = 1), sex (males n = 421; females n = 329; 3 unidentified), and age (juvenile n = 199; after-hatch-year n = 549; 5 unidentified). Of these 753 hummingbirds evaluated, mites were present on the rectrices of 40.9% of the birds. Significantly more Anna's Hummingbirds were positive for rectricial mites (59.2%) compared with 8.2% of Black-chinned, 0.9% of Allen's, 5.6% of Rufous Hummingbirds, and 0% for Calliope (p-value < 0.0001). Across all hummingbird species, male hummingbirds (44.9%) had a higher prevalence of rectricial mites compared to female hummingbirds (36.2%; p-value = 0.004), while juvenile hummingbirds (46.2%) had a non-significantly higher prevalence compared to after-hatch-year hummingbirds (39.0%; p-value = 0.089). On average, the percentage of the long axis of the rachis occupied by mites for the outer rectrices (R4 and R5) was 19%, compared to 11% for inner rectrices (R1 and R2), a significant difference (p-value = <0.0001). There was a marginal lack of significance for symmetrical distribution of tail mites with the mean left side percentage of long axis of the rachis occupied by mites being 16% and very close to the mean right side score of 18% (p-value = 0.003). The identification of the feather mite species was based on light microscopic morphometry, and mite distribution on feathers was further evaluated using tabletop scanning electron microscopy (TSEM). The hummingbird-feather mite relationship is not well understood, but the specialized TSEM technique may be especially useful in examining natural positioning and developmental aspects of the mites since it allows in situ feather examination of live mites.


Asunto(s)
Aves/parasitología , Plumas/parasitología , Ácaros/clasificación , Animales , California , Femenino , Vuelo Animal , Masculino , Microscopía/métodos , Microscopía Electrónica de Rastreo/métodos , Ácaros/genética , Ácaros/patogenicidad , Prevalencia
3.
Am J Clin Pathol ; 140(2): 170-6, 2013 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-23897251

RESUMEN

OBJECTIVES: Intracellular components of transfusion-related acute lung injury (TRALI) were investigated by transmission electron microscopy. METHODS: The lungs from 2 fatal TRALI cases and 2 controls, previously studied by scanning electron microscopy, were studied by transmission electron microscopy. Morphologic data by light and phase microscopy, along with scanning and transmission electron microscopic observations, were collated. RESULTS: The 2 fatal TRALI cases exhibited dense laminated material within capillaries and postcapillary venules, similar to material identified within their neutrophils when viewed by transmission electron microscopy. This material polarized light and is presumed to be cholesterol crystals. CONCLUSIONS: The damage to the pulmonary vascular endothelium in TRALI is related to formation of cholesterol crystals originating within neutrophils.


Asunto(s)
Lesión Pulmonar Aguda/sangre , Colesterol/metabolismo , Neutrófilos/metabolismo , Reacción a la Transfusión , Lesión Pulmonar Aguda/etiología , Lesión Pulmonar Aguda/metabolismo , Adulto , Humanos , Masculino
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