Open AccessMonomethylfumarate Induces γ-Globin Expression and Fetal Hemoglobin Production in Cultured Human Retinal Pigment Epithelial (RPE) and Erythroid Cells, and in Intact Retina
Author(s) -
Wanwisa Promsote,
Levi Makala,
Biaoru Li,
Sylvia B. Smith,
Nagendra Singh,
Vadivel Ganapathy,
Betty S. Pace,
Pamela M. Martin
Publication year - 2014
Publication title -
investigative ophthalmology and visual science
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.935
H-Index - 218
eISSN - 1552-5783
pISSN - 0146-0404
DOI - 10.1167/iovs.14-14179
Subject(s) - fetal hemoglobin , microbiology and biotechnology , biology , retinal , retina , retinal pigment epithelium , cell culture , globin , western blot , hemoglobin , fetus , biochemistry , gene , genetics , pregnancy , neuroscience
Sickle retinopathy (SR) is a major cause of vision loss in sickle cell disease (SCD). There are no strategies to prevent SR and treatments are extremely limited. The present study evaluated (1) the retinal pigment epithelial (RPE) cell as a hemoglobin producer and novel cellular target for fetal hemoglobin (HbF) induction, and (2) monomethylfumarate (MMF) as an HbF-inducing therapy and abrogator of oxidative stress and inflammation in SCD retina.