Phenotypic and functional characterization of Bst+/- mouse retina

Hamidreza Riazifar, Guoli Sun, Xinjian Wang, Alan Rupp, Shruti Vemaraju, Fred N. Ross-Cisneros, Richard A. Lang, Alfredo A. Sadun, Samer Hattar, Min Xin Guan, Taosheng Huang

Research output: Contribution to journalArticle

Abstract

The belly spot and tail (Bst+/-) mouse phenotype is caused by mutations of the ribosomal protein L24 (Rpl24). Among various phenotypes in Bst+/- mice, the most interesting are its retinal abnormalities, consisting of delayed closure of choroid fissures, decreased ganglion cells and subretinal vascularization. We further characterized the Bst+/- mouse and investigated the underlying molecular mechanisms to assess the feasibility of using this strain as a model for stem cell therapy of retinal degenerative diseases due to retinal ganglion cell (RGC) loss. We found that, although RGCs are significantly reduced in retinal ganglion cell layer in Bst+/- mouse, melanopsin+ RGCs, also called ipRGCs, appear to be unchanged. Pupillary light reflex was completely absent in Bst+/- mice but they had a normal circadian rhythm. In order to examine the pathological abnormalities in Bst+/- mice, we performed electron microscopy in RGC and found that mitochondria morphology was deformed, having irregular borders and lacking cristae. The complex activities of the mitochondrial electron transport chain were significantly decreased. Finally, for subretinal vascularization, we also found that angiogenesis is delayed in Bst+/- associated with delayed hyaloid regression. Characterization of Bst+/- retina suggests that the Bst+/- mouse strain could be a useful murine model. It might be used to explore further the pathogenesis and strategy of treatment of retinal degenerative diseases by employing stem cell technology.

Original languageEnglish (US)
Pages (from-to)969-976
Number of pages8
JournalDMM Disease Models and Mechanisms
Volume8
Issue number8
DOIs
StatePublished - Aug 1 2015

Fingerprint

Stem cells
Retina
Mitochondria
Retinal Ganglion Cells
Electron microscopy
Cells
Retinal Diseases
Stem Cells
Pupillary Reflex
Phenotype
Choroid
Cell- and Tissue-Based Therapy
Electron Transport
Circadian Rhythm
Ganglia
Tail
Electron Microscopy
Technology
Light
Mutation

Keywords

  • Bst
  • Melanopsin
  • Retinal ganglion cell

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)
  • Medicine (miscellaneous)
  • Immunology and Microbiology (miscellaneous)
  • Neuroscience (miscellaneous)

Cite this

Riazifar, H., Sun, G., Wang, X., Rupp, A., Vemaraju, S., Ross-Cisneros, F. N., ... Huang, T. (2015). Phenotypic and functional characterization of Bst+/- mouse retina. DMM Disease Models and Mechanisms, 8(8), 969-976. https://doi.org/10.1242/dmm.018176

Phenotypic and functional characterization of Bst+/- mouse retina. / Riazifar, Hamidreza; Sun, Guoli; Wang, Xinjian; Rupp, Alan; Vemaraju, Shruti; Ross-Cisneros, Fred N.; Lang, Richard A.; Sadun, Alfredo A.; Hattar, Samer; Guan, Min Xin; Huang, Taosheng.

In: DMM Disease Models and Mechanisms, Vol. 8, No. 8, 01.08.2015, p. 969-976.

Research output: Contribution to journalArticle

Riazifar, H, Sun, G, Wang, X, Rupp, A, Vemaraju, S, Ross-Cisneros, FN, Lang, RA, Sadun, AA, Hattar, S, Guan, MX & Huang, T 2015, 'Phenotypic and functional characterization of Bst+/- mouse retina', DMM Disease Models and Mechanisms, vol. 8, no. 8, pp. 969-976. https://doi.org/10.1242/dmm.018176
Riazifar H, Sun G, Wang X, Rupp A, Vemaraju S, Ross-Cisneros FN et al. Phenotypic and functional characterization of Bst+/- mouse retina. DMM Disease Models and Mechanisms. 2015 Aug 1;8(8):969-976. https://doi.org/10.1242/dmm.018176
Riazifar, Hamidreza ; Sun, Guoli ; Wang, Xinjian ; Rupp, Alan ; Vemaraju, Shruti ; Ross-Cisneros, Fred N. ; Lang, Richard A. ; Sadun, Alfredo A. ; Hattar, Samer ; Guan, Min Xin ; Huang, Taosheng. / Phenotypic and functional characterization of Bst+/- mouse retina. In: DMM Disease Models and Mechanisms. 2015 ; Vol. 8, No. 8. pp. 969-976.
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