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Biochimica et biophysica acta
Jan, 2016;1860 (1 Pt B): 287-98.

βA3/A1-crystallin and persistent fetal vasculature (PFV) disease of the eye.

J Samuel Zigler, Mallika Valapala, Peng Shang, Stacey Hose, Morton F Goldberg, Debasish Sinha
Author Information
  1. J Samuel Zigler: Wilmer Eye Institute, The Johns Hopkins University School of Medicine, Baltimore, MD, USA.
  2. Mallika Valapala: Wilmer Eye Institute, The Johns Hopkins University School of Medicine, Baltimore, MD, USA.
  3. Peng Shang: Wilmer Eye Institute, The Johns Hopkins University School of Medicine, Baltimore, MD, USA; Department of Ophthalmology of Shanghai Tenth Hospital and Tongji Eye Institute, Tongji University School of Medicine, Shanghai, China.
  4. Stacey Hose: Wilmer Eye Institute, The Johns Hopkins University School of Medicine, Baltimore, MD, USA.
  5. Morton F Goldberg: Wilmer Eye Institute, The Johns Hopkins University School of Medicine, Baltimore, MD, USA.
  6. Debasish Sinha: Wilmer Eye Institute, The Johns Hopkins University School of Medicine, Baltimore, MD, USA. Electronic address: Debasish@jhmi.edu.
PMID: 26022148 DOI: 10.1016/j.bbagen.2015.05.017

Abstract

BACKGROUND: Persistent fetal vasculature (PFV) is a human disease in which the fetal vasculature of the eye fails to regress normally. The fetal, or hyaloid, vasculature nourishes the lens and retina during ocular development, subsequently regressing after formation of the retinal vessels. PFV causes serious congenital pathologies and is responsible for as much as 5% of blindness in the United States.
SCOPE OF REVIEW: The causes of PFV are poorly understood, however there are a number of animal models in which aspects of the disease are present. One such model results from mutation or elimination of the gene (Cryba1) encoding βA3/A1-crystallin. In this review we focus on the possible mechanisms whereby loss of functional βA3/A1-crystallin might lead to PFV.
MAJOR CONCLUSIONS: Cryba1 is abundantly expressed in the lens, but is also expressed in certain other ocular cells, including astrocytes. In animal models lacking βA3/A1-crystallin, astrocyte numbers are increased and they migrate abnormally from the retina to ensheath the persistent hyaloid artery. Evidence is presented that the absence of functional βA3/A1-crystallin causes failure of the normal acidification of endolysosomal compartments in the astrocytes, leading to impairment of certain critical signaling pathways, including mTOR and Notch/STAT3.
GENERAL SIGNIFICANCE: The findings suggest that impaired endolysosomal signaling in ocular astrocytes can cause PFV disease, by adversely affecting the vascular remodeling processes essential to ocular development, including regression of the fetal vasculature. This article is part of a Special Issue entitled Crystallin Biochemistry in Health and Disease.

Keywords

Astrocytes Fetal/hyaloid vasculature Notch/STAT signaling PI3K/Akt/mTOR signaling Vascular remodeling βA3/A1-crystallin

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Grants

  1. EY019037/NEI NIH HHS
  2. R01 EY019037/NEI NIH HHS
  3. P30 EY001765/NEI NIH HHS
  4. EY018636/NEI NIH HHS
  5. R01 EY018636/NEI NIH HHS
  6. EY01765/NEI NIH HHS
  7. EY019037-S/NEI NIH HHS

MeSH Term

Animals
Chronic Disease
Eye Proteins
Humans
Models, Biological
Persistent Hyperplastic Primary Vitreous
Retinal Vessels
beta-Crystallin A Chain

Chemicals

CRYBA1 protein, human
Eye Proteins
beta-Crystallin A Chain
Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Review

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