Diagnosing papillorenal syndrome: see the optic papilla

Parsa, Cameron F.; Parsa, Afshin

doi:10.1007/s00467-008-0870-6

Cited by 12 publications

(9 citation statements)

References 9 publications

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“…Lack of vascularization in the central retina was only observed in Bcl-2 PC mice, where vessels appeared to enter and bifurcate near the disc periphery. Such aberrant vascularization in the central retina is similar to that observed in papillorenal syndrome in which retinas lack central vessels that should have emerged from the periphery of the nerve papilla as well as renal hypoplasia 36–38 , as we observed in global Bcl-2 −/− mice. However, unlike the global Bcl-2 −/− mice we did not observe renal hypoplasia in Bcl-2 PC mice as evidenced by similar kidney to body weight ratios of 3 week old Bcl-2 Flox/Flox and Bcl-2 PC mice (Fig.…”

Section: Discussionsupporting

confidence: 83%

Bcl-2 Expression in Pericytes and Astrocytes Impacts Vascular Development and Homeostasis

Zaitoun

Wintheiser

Jamali

et al. 2019

Sci Rep

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B-cell lymphoma 2 (Bcl-2) protein is the founding member of a group of proteins known to modulate apoptosis. Its discovery set the stage for identification of family members with either pro- or anti-apoptotic properties. Expression of Bcl-2 plays an important role during angiogenesis by influencing not only vascular cell survival, but also migration and adhesion. Although apoptosis and migration are postulated to have roles during vascular remodeling and regression, the contribution of Bcl-2 continues to emerge. We previously noted that the impaired retinal vascularization and an inability to undergo pathologic neovascularization observed in mice globally lacking Bcl-2 did not occur when mice lacked the expression of Bcl-2 only in endothelial cells. To further examine the effect of Bcl-2 expression during vascularization of the retina, we assessed its contribution in pericytes or astrocytes by generating mice with a conditional Bcl-2 allele (Bcl-2 Flox/Flox ) and Pdgfrb-cre (Bcl-2 PC mice) or Gfap-cre (Bcl-2 AC mice). Bcl-2 PC and Bcl-2 AC mice demonstrated increased retinal vascular cell apoptosis, reduced numbers of pericytes and endothelial cells and fewer arteries and veins in the retina. Bcl-2 PC mice also demonstrated delayed advancement of the superficial retinal vascular layer and aberrant vascularization of the deep vascular plexus and central retina. Although pathologic neovascularization in oxygen-induced ischemic retinopathy (OIR) was not affected by lack of expression of Bcl-2 in either pericytes or astrocytes, laser-induced choroidal neovascularization (CNV) was significantly reduced in Bcl-2 PC mice compared to littermate controls. Together these studies begin to reveal how cell autonomous modulation of apoptosis in vascular cells impacts development and homeostasis.

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Section: Discussionsupporting

confidence: 83%

Bcl-2 Expression in Pericytes and Astrocytes Impacts Vascular Development and Homeostasis

Zaitoun

Wintheiser

Jamali

et al. 2019

Sci Rep

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“…Iran's revolution against a monarchy in 1979 occurred in large part due to the financial and mobilization efforts of firm owners and entrepreneurs among the country's bazaar sectors. 103 Saudi Arabia's experience with business environment reforms also leads us to question the long-standing argument about a "king's dilemma," whereby monarchies are inherently unable to pursue modernization without risking their own downfall. 104 Although the concept of "modernization" has lost its appeal among scholars in recent years, to the extent that business environment reforms are one facet of "economic modernization," we can conclude that Saudi Arabia is an exception to the king's dilemma in this respect.…”

Section: Resultsmentioning

confidence: 99%

The Saudi monarchy and economic familism in an era of business environment reforms

Mazaheri¹

2013

Bus. polit.

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In recent years, Saudi Arabia has strengthened its regulatory and financial institutions and adopted many reforms concerning its business environment. Yet, Saudi Arabia seems an unlikely country to succeed at implementing business environment reforms given the presence of an authoritarian state and rent-seeking behavior from elites that is the outcome of oil wealth. What explains the ability of Saudi Arabia to initiate reforms that many states have struggled to implement or uniformly reject? This paper argues that the country's monarchical system helps the government solve the credible commitment problem with private sector elites, thereby facilitating business environment reforms. The monarchical system does this by legitimizing and reinforcing the institution of economic familism. The salience of this institution provides a reliable guarantee to private sector elites that their rents and business interests will be protected during the reform process. The case of Saudi Arabia stands as an important example of how absolute monarchies can pursue certain economic reforms, and also how an informal institution can solve the credible commitment problem in an authoritarian context where formal institutions are either absent or weak.

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“…45 Other mutations that cause systemic inhibition of angiogenesis are known to produce a different constellation of findings, with pathognomonic vascular findings visible at the level of the optic disc. [46][47][48]…”

Section: Etiology Of Duane Syndrome and Benign Recurrent Sixth Nerve mentioning

confidence: 99%

Thromboembolism and Congenital Malformations

Parsa

Robert²

2013

JAMA Ophthalmol

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To propose a pathophysiologic mechanism to unify a variety of disparate sporadic congenital malformations. Methods: Inductive and deductive analyses to correlate malformation laterality with asymmetries in thoracic anatomy, critical analysis of malformations with female predominance, and concepts of hydrodynamic pressure gradients in vascular growth were applied to the ensuing development of guiding tissue scaffolds for cellular proliferation, differentiation, and apoptosis. Results: Duane syndrome may develop following a focal vascular insult to the sixth nerve trunk with axonal degeneration, allowing for substitutive innervation from third nerve axons to the lateral rectus muscle. Causative fibrin clots may originate from the venous system and paradoxically migrate through physiological right-toleft shunts, or they may arise directly from the heart. Hence, the unilateral, left-sided, and female predominance of Duane syndrome results from the asymmetry in the thoracic anatomy and from thrombosis risk factors. Embolic occlusions may also alter local hemody-namic pressure gradients, leading to the compensatory enlargement and persistence of the fetal vasculature and may dysregulate tissue growth. Within the eye, this results in forms of Peters anomaly, unilateral congenital cataracts, and the morning glory disc anomaly, all in the vascular territory of the carotid arteries that also share a propensity for left-sided involvement in girls. Most aberrant misinnervation phenomena (eg, jaw-winking syndrome, crocodile tear syndrome, Brown syndrome, and congenital fibrosis syndrome) and, by extrapolation, the hypoplasia or dysgenesis of noncephalic anatomical structures (including limbs) may be similarly explained. Such malformations will occur more frequently under thrombogenic conditions, such as those induced by thalidomide. Conclusions: Fibrin emboli and focal hypoperfusion may explain the development of many sporadic congenital malformations.

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Diagnosing papillorenal syndrome: see the optic papilla

Cited by 12 publications

References 9 publications

Bcl-2 Expression in Pericytes and Astrocytes Impacts Vascular Development and Homeostasis

Bcl-2 Expression in Pericytes and Astrocytes Impacts Vascular Development and Homeostasis

The Saudi monarchy and economic familism in an era of business environment reforms

Thromboembolism and Congenital Malformations

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