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Retinal Metabolic Profile on IMPG2 Deficiency Mice with Subretinal Lesions
Rong Xu1, Yekai Wang1, Jianhai Du1
1Departments of Biochemistry and molecular medicine, and Ophthalmology and Visual Sciences, West Virginia University, Morgantown, WV, USA.
Advances in Experimental Medicine and Biology
|July 13, 2023
Summary
Mutations in IMPG2 cause vision-impairing lesions. IMPG2 knockout mice show reduced glutamate, urea, and galactose metabolism, indicating impaired photoreceptor energy metabolism in these models.
Area of Science:
- Ophthalmology
- Molecular Biology
- Biochemistry
Background:
- The interphotoreceptor matrix (IPM) is crucial for photoreceptor and retinal pigment epithelium (RPE) function.
- Mutations in interphotoreceptor matrix proteoglycan 2 (IMPG2) lead to subretinal vitelliform lesions and vision impairment.
- IMPG2 knockout (KO) mice models exhibit similar subretinal lesions and mislocalized IMPG1.
Purpose of the Study:
- To investigate the impact of subretinal material accumulation on photoreceptor metabolism in IMPG2 KO mice.
- To analyze metabolic alterations in the retinas of IMPG2 KO mice using targeted metabolomics.
Main Methods:
- Generation of IMPG2 knockout (KO) mice exhibiting subretinal lesions.
- Targeted metabolomics to profile the retinal metabolome of IMPG2 KO mice.
- Metabolite set enrichment analysis to identify affected metabolic pathways.
Main Results:
- IMPG2 KO mice retinas display subretinal lesions partly composed of mislocalized IMPG1.
- Microscopic IMPG1 accumulation occurs between the RPE and photoreceptor outer segments in IMPG2 KO mice.
- Reduced glutamate metabolism, urea cycle, and galactose metabolism were observed in IMPG2 KO mice retinas.
Conclusions:
- Subretinal material accumulation in IMPG2 KO mice retinas is associated with altered photoreceptor metabolism.
- The observed metabolic changes suggest impaired energy metabolism in IMPG2-deficient retinas with subretinal lesions.
- These findings highlight the role of IMPG2 in maintaining retinal metabolic homeostasis.
