Leber hereditary optic neuropathy (LHON) is a disorder of the retina and optic nerve associated with a mutation in mitochondrial DNA (mtDNA). Developing therapies for disorders caused by mtDNA mutations is challenging because of a lack of suitable animal models.
Researchers in the journal PNAS. introduced human mtDNA containing the LHON mutation directly into mitochondria of mouse zygotes using a virus. The zygotes developed into transgenic mice that expressed the mutant gene in their optic nerves and retinas.
Breeding transgenic females with nontransgenic males for several generations produced more than 200 transgenic mice exhibiting hallmark signs of LHON, including progressive vision loss, thinning of the retina, and severe optic nerve atrophy.
Transgenic mice also exhibited increased oxidative stress and decreased cellular respiration in the optic nerve, compared with control mice. The authors were able to partially reverse the vision loss by injecting a virus containing wild-type human mtDNA into the eyes of the transgenic mice.
The authors suggest that their method could be adapted to create mouse models of other disorders caused by mtDNA mutations, enabling tests of treatments for such disorders. The results also suggest that gene therapy might be a promising approach to treat such disorders.
http://www.pnas.org/content/early/2015/09/30/1506129112
Mouse model of mitochondrial DNA mutations
- 2,061 views
- Added
Edited
Latest News
Maternal obesity may promot…
By newseditor
Posted 30 May
The neuronal basis of opioi…
By newseditor
Posted 30 May
Circadian clock can be leve…
By newseditor
Posted 30 May
Connecting genetic risk for…
By newseditor
Posted 30 May
Tumor infiltration of immun…
By newseditor
Posted 28 May
Other Top Stories
A new mechanism for bacterial division
Read more
Gut bacteria can help to predict how the body will respond to fatty…
Read more
Simple blood test predicts anemia risk after malaria treatment
Read more
Antibiotics taken late in pregnancy can increase risk for inflammat…
Read more
Storing and replaying a movie encoded in bacterial genome
Read more
Protocols
Accessible high-speed image…
By newseditor
Posted 30 May
SEMORE: SEgmentation and MO…
By newseditor
Posted 26 May
Spatially resolved lipidomi…
By newseditor
Posted 24 May
Efficient expansion and CRI…
By newseditor
Posted 21 May
Massively parallel in vivo…
By newseditor
Posted 20 May
Publications
Maternal obesity increases…
By newseditor
Posted 30 May
Why cells need iron: a comp…
By newseditor
Posted 30 May
Distinct μ-opioid ensembles…
By newseditor
Posted 30 May
Axonal endoplasmic reticulu…
By newseditor
Posted 30 May
I Am Curious About Cannabis…
By newseditor
Posted 30 May
Presentations
Hydrogels in Drug Delivery
By newseditor
Posted 12 Apr
Lipids
By newseditor
Posted 31 Dec
Cell biology of carbohydrat…
By newseditor
Posted 29 Nov
RNA interference (RNAi)
By newseditor
Posted 23 Oct
RNA structure and functions
By newseditor
Posted 19 Oct
Posters
A chemical biology/modular…
By newseditor
Posted 22 Aug
Single-molecule covalent ma…
By newseditor
Posted 04 Jul
ASCO-2020-HEALTH SERVICES R…
By newseditor
Posted 23 Mar
ASCO-2020-HEAD AND NECK CANCER
By newseditor
Posted 23 Mar
ASCO-2020-GENITOURINARY CAN…
By newseditor
Posted 23 Mar