Data on mitochondrial ultrastructure of photoreceptors in pig, rabbit, and mouse retinas

Ozaki, Taku; Utsumi, Shinto; Iwamoto, Takeshi; Tanaka, Makoto; Tomita, Hiroshi; Sugano, Eriko; Ishiyama, Eri; Ishida, Katsuaki

doi:10.1016/j.dib.2020.105544

Cited by 3 publications

(3 citation statements)

References 12 publications

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“…Cone PR morphology and distribution are 2 main identifying characteristics in AO images ( 51 – 53 ). Pig cone PR diameter in our study was within the range of previously reported human cone PR diameter (3.0 ± 0.4 μm to 8.2 ± 0.6 μm; mean ± SD) ( 54 , 55 ). In our data, the cone PR density at the baseline was consistent with Huckenpahler ( 51 ) and comparable with spacing and density of human cones PRs imaged at 4° eccentricity using the same AO system used in our study ( 52 ).…”

Section: Discussionsupporting

confidence: 87%

A versatile laser-induced porcine model of outer retinal and choroidal degeneration for preclinical testing

Barone¹,

Amaral²,

Bunea³

et al. 2023

JCI Insight

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Over 30 million people worldwide suffer from untreatable vision loss and blindness associated with childhood-onset and age-related eye diseases caused by photoreceptor (PR), retinal pigment epithelium (RPE), and choriocapillaris (CC) degeneration. Recent work suggests that RPE-based cell therapy may slow down vision loss in late stages of age-related macular degeneration (AMD), a polygenic disease induced by RPE atrophy. However, accelerated development of effective cell therapies is hampered by the lack of large-animal models that allow testing safety and efficacy of clinical doses covering the human macula (20 mm 2 ). We developed a versatile pig model to mimic different types and stages of retinal degeneration. Using an adjustable power micropulse laser, we generated varying degrees of RPE, PR, and CC damage and confirmed the damage by longitudinal analysis of clinically relevant outcomes, including analyses by adaptive optics and optical coherence tomography/angiography, along with automated image analysis. By imparting a tunable yet targeted damage to the porcine CC and visual streak — with a structure similar to the human macula — this model is optimal for testing cell and gene therapies for outer retinal diseases including AMD, retinitis pigmentosa, Stargardt, and choroideremia. The amenability of this model to clinically relevant imaging outcomes will facilitate faster translation to patients.

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Section: Discussionsupporting

confidence: 87%

A versatile laser-induced porcine model of outer retinal and choroidal degeneration for preclinical testing

Barone¹,

Amaral²,

Bunea³

et al. 2023

JCI Insight

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“…Mitochondrial number was determined using FEI software. Mitochondrial surface area and perimeter were analyzed on the same images using the iTEM software, and these data are represented normalized per field, as previously reported (Costa et al , 2010; Naon et al , 2016; Quintana‐Cabrera et al , 2018; Indrieri et al , 2019; Tsakiri et al , 2019; Xia et al , 2019; Ozaki et al , 2020; Anastasia et al , 2021; Mirra et al , 2021). The researchers in charge of producing the TEM images were blind to the genotypes of the animals.…”

Section: Methodsmentioning

confidence: 99%

miR ‐181a/b downregulation: a mutation‐independent therapeutic approach for inherited retinal diseases

et al. 2022

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Inherited retinal diseases (IRDs) are a group of diseases whose common landmark is progressive photoreceptor loss. The development of gene-specific therapies for IRDs is hampered by their wide genetic heterogeneity. Mitochondrial dysfunction is proving to constitute one of the key pathogenic events in IRDs; hence, approaches that enhance mitochondrial activities have a promising therapeutic potential for these conditions. We previously reported that miR-181a/b downregulation boosts mitochondrial turnover in models of primary retinal mitochondrial diseases. Here, we show that miR-181a/b silencing has a beneficial effect also in IRDs. In particular, the injection in the subretinal space of an adeno-associated viral vector (AAV) that harbors a miR-181a/b inhibitor (sponge) sequence (AAV2/8-GFP-Sponge-miR-181a/b) improves retinal morphology and visual function both in models of autosomal dominant (RHO-P347S) and of autosomal recessive (rd10) retinitis pigmentosa. Moreover, we demonstrate that miR-181a/b downregulation modulates the level of the mitochondrial fission-related protein Drp1 and rescues the mitochondrial fragmentation in RHO-P347S photoreceptors. Overall, these data support the potential use of miR-181a/b downregulation as an innovative mutation-independent therapeutic strategy for IRDs, which can be effective both to delay disease progression and to aid gene-specific therapeutic approaches.

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“…TEM was performed to compare the mitochondrial morphological features of WT and ES1 -KO mice. The method of sampling was the same as that reported by Ozaki et al [35] . The brains were obtained from WT and ES1 -KO mice and stored in a fixative solution (2.5% glutaraldehyde, 2% paraformaldehyde, 0.1 M sodium phosphate buffer, pH 7.4) overnight at 4°C.…”

Section: Methodsmentioning

confidence: 99%

Novel dicarbonyl metabolic pathway via mitochondrial ES1 possessing glyoxalase III activity

et al. 2023

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Data on mitochondrial ultrastructure of photoreceptors in pig, rabbit, and mouse retinas

Cited by 3 publications

References 12 publications

A versatile laser-induced porcine model of outer retinal and choroidal degeneration for preclinical testing

A versatile laser-induced porcine model of outer retinal and choroidal degeneration for preclinical testing

miR ‐181a/b downregulation: a mutation‐independent therapeutic approach for inherited retinal diseases

Novel dicarbonyl metabolic pathway via mitochondrial ES1 possessing glyoxalase III activity

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