Refractive Changes Induced by Form Deprivation in the Mouse Eye

Tejedor, Jaime; Villa, Pedro de la

doi:10.1167/iovs.01-1171

Cited by 109 publications

(76 citation statements)

References 24 publications

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“…The spherical equivalent retinoscopic refraction in control mice was 13.89 ± 0.85 diopters (mean ± standard deviation); in H258N mice, it was 17.71±0.86 diopters. The hyperopic difference corresponded appropriately to a decreased eye axial length (data not shown) [Tejedor and de la Villa, 2003]. Thus, the mouse model exhibits a refractive error distinct from that seen in human CSNB.…”

Section: Phenotypic Analyses Of H258n Micementioning

confidence: 80%

“…While emmetropization occurs in most normal individuals by early childhood, a myopic refractive error persists in CSNB patients. To determine whether H258N mice displayed similar features, streak retinoscopy was performed as previously described (Tejedor and de la Villa, 2003]. The spherical equivalent retinoscopic refraction in control mice was 13.89 ± 0.85 diopters (mean ± standard deviation); in H258N mice, it was 17.71±0.86 diopters.…”

Section: Phenotypic Analyses Of H258n Micementioning

confidence: 99%

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Transgenic mice carrying theH258N mutation in the gene encoding the β-subunit of phosphodiesterase-6 (PDE6B) provide a model for human congenital stationary night blindness

Tsang

Woodruff

et al. 2007

Hum. Mutat.

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Mutations in the β-subunit of cGMP-phosphodiesterase (PDE6β) can lead to either progressive retinal disease, such as human retinitis pigmentosa (RP), or stationary disease, such as congenital stationary night blindness (CSNB). Individuals with CSNB in the Rambusch pedigree were found to carry the H258N allele of PDE6B (MIM# 180072); a similar mutation was not found in RP patients. This report describes an individual carrying the H258N allele, who presented with generalized retinal dysfunction affecting the rod system and a locus of dysfunction at the rod-bipolar interface. Also described are preclinical studies in which transgenic mice with the H258N allele were generated to study the pathophysiological mechanisms of CSNB. While Pde6b rd1 /Pde6b rd1 mice have severe photoreceptor degeneration, as in human RP, the H258N transgene rescued these cells. The cGMP-PDE6 activity of dark-adapted H258N mice showed an approximate three-fold increase in the rate of retinal cGMP hydrolysis: from 130.1 nmol × min −1 × nmol −1 rhodopsin in wild-type controls to 319.2 nmol × min −1 × nmol −1 rhodopsin in mutants, consistent with the hypothesis that inhibition of the PDE6β activity by the regulatory PDE6γ subunit is blocked by this mutation. In the albino (B6CBA × FVB) F2 hybrid background, electroretinograms (ERG) from H258N mice were similar to those obtained from affected Rambusch family members, as well as humans with the most common form of CSNB (X-linked), demonstrating a selective loss of the b-wave with relatively normal awaves. When the H258N allele was introduced into the DBA background, there was no evidence of selective reduction in b-wave amplitudes; rather a-and b-wave amplitudes were both reduced. Thus, factors other than the PDE6B mutation itself could contribute to the variance of an

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Section: Phenotypic Analyses Of H258n Micementioning

confidence: 80%

Section: Phenotypic Analyses Of H258n Micementioning

confidence: 99%

Transgenic mice carrying theH258N mutation in the gene encoding the β-subunit of phosphodiesterase-6 (PDE6B) provide a model for human congenital stationary night blindness

Tsang

Woodruff

et al. 2007

Hum. Mutat.

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“…Importantly, the postnatal differentiation is most likely the result of proliferative demands on the corneal keratocytes to increase corneal thickness and control corneal shape for the proper refraction of light at the time of eyelid opening. The role of light in modifying this last stage of differentiation remains to be clarified, and whether eyelid opening results in feedback control from the retina to the cornea as occurs in the sclera during the development of form-deprivation myopia [75] is unknown and needs further study.…”

Section: Developmental Regulation Of Transparency and Expression Of Cmentioning

confidence: 99%

Corneal crystallins and the development of cellular transparency

Jester

2008

Seminars in Cell & Developmental Biology

161

129

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Past studies have established that the cornea like the lens abundantly expresses a few water-soluble enzyme/proteins in a taxon specific fashion. Based on these similarities it has been proposed that the lens and the cornea form a structural unit the 'refracton' that has co-evolved through gene sharing to maximize light transmission and refraction to the retina. Thus far, the analogy between corneal crystallins and lens crystallins has been limited to similarities in the abundant expression, with few reports concerning their structural function. This review covers recent studies that establish a clear relationship between expression of corneal crystallins and light scattering from corneal stromal cells, i.e. keratocytes, that support a structural role for corneal crystallins in the development of transparency similar to that of lens crystallins that would be consistent with the 'refracton' hypothesis.

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“…In recent years, the use of the mouse in myopia research has been attempted, but with some difficulty. Several groups have attempted to induce experimental myopia in mice via form deprivation using methods such as lid suture (Tejedor and de la Villa, 2003) and gluing a goggle to the fur surrounding the eye . There are advantages and disadvantages associated with both methods; however, neither is ideal.…”

Section: Introductionmentioning

confidence: 99%