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Regenerative Medicine and Stem Cell Therapy for the Eye pp 123–148Cite as

Limbal Stem Cells and the Treatment of Limbal Stem Cell Deficiency

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Part of the book series: Fundamental Biomedical Technologies ((FBMT))

Abstract

Many organs (skin, stomach, intestines, colon, and eye) possess an epithelial layer that is short-lived and rapidly lost, requiring a source of adult stem cells that produces a continual supply of epithelial cells to replenish the lost cells. Maintaining these rapidly self-renewing epithelial surfaces during normal homeostasis is therefore dependent upon the health of the adult stem cell population. One important challenge in regenerative medicine is replacing these adult stem cells when they are eliminated following an injury or disease. The eye contains two highly specialized stratified squamous epithelia, the conjunctival epithelium and the corneal epithelium, which are separated by the limbal epithelium (Fig. 5.1). A healthy corneal epithelium is essential for maintaining a clear cornea and normal vision. The limbus contains a small subpopulation of rare LSC (Limbal Stem Cells) that continually repopulates the corneal epithelium. Patients with a LSCD (Limbal Stem Cell Deficiency) are unable to regenerate the corneal epithelium, resulting in migration of the conjunctival epithelium over the corneal stroma, called “conjunctivalization,” that triggers neovascularization, chronic inflammation, and corneal opacity. A complete LSCD results in the total loss of the corneal epithelium and blindness due to an irreversibly opaque cornea. The extent of LSC loss can range from partial to complete and can be either unilateral or bilateral with a corresponding range in the loss of vision. LSCD can be caused by a variety of injuries or diseases: chemical or thermal burns [1], Stevens-Johnson syndrome [2, 3], aniridia [3], contact lens-induced keratopathy [4], multiple surgeries [5], cryotherapy of the limbus [5], chronic peripheral corneal inflammation [6], and lysosomal storage disease. However, corneal burns are by far the most frequent cause of a LSCD [5].

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Authors and Affiliations

  1. Schepens Eye Research Institute, Massachusetts Eye and Ear Infirmary, Boston, MA, USA

    Bruce R. Ksander

  2. Transplantation Research Center, Boston Children’s Hospital, Boston, MA, USA

    Markus H. Frank

  3. Department of Dermatology, Brigham and Women’s Hospital, Boston, MA, USA

    Markus H. Frank

  4. Department of Medicine, VA Boston Healthcare System, Harvard Medical School, Boston, MA, USA

    Natasha Y. Frank

  5. Division of Genetics, Brigham and Women’s Hospital, Boston, MA, USA

    Natasha Y. Frank

Authors
  1. Bruce R. Ksander
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  2. Markus H. Frank
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  3. Natasha Y. Frank
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Correspondence to Bruce R. Ksander .

Editor information

Editors and Affiliations

  1. Department Ophthalmology and Vision Sciences, University of Toronto, Toronto, ON, Canada

    Brian G. Ballios

  2. Schepens Eye Research Institute, Harvard Medical School, Boston, MA, USA

    Michael J. Young

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Ksander, B.R., Frank, M.H., Frank, N.Y. (2018). Limbal Stem Cells and the Treatment of Limbal Stem Cell Deficiency. In: Ballios, B., Young, M. (eds) Regenerative Medicine and Stem Cell Therapy for the Eye. Fundamental Biomedical Technologies. Springer, Cham. https://doi.org/10.1007/978-3-319-98080-5_5

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