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Visual evoked potentials of Niemann-Pick type C1 mice reveal an impairment of the visual pathway that is rescued by 2-hydroxypropyl-ß-cyclodextrin

Overview of attention for article published in Orphanet Journal of Rare Diseases, October 2015
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17 Dimensions

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Title
Visual evoked potentials of Niemann-Pick type C1 mice reveal an impairment of the visual pathway that is rescued by 2-hydroxypropyl-ß-cyclodextrin
Published in
Orphanet Journal of Rare Diseases, October 2015
DOI 10.1186/s13023-015-0348-0
Pubmed ID
Authors

Giampiero Palladino, Stefano Loizzo, Andrea Fortuna, Sonia Canterini, Fioretta Palombi, Robert P. Erickson, Franco Mangia, Maria Teresa Fiorenza

Abstract

The lysosomal storage disorder, Niemann Pick type C1 (NPC1), presents a variable phenotype including neurovisceral and neurological symptoms. 2-Hydroxypropyl-ß-cyclodextrin (HPßCD)-based therapies are presently the most promising route of intervention. While severe cerebellar dysfunction remains the main disabling feature of NPC1, sensory functions including auditory and olfactory ones are also affected. Morphological and functional anomalies of Npc1 (-/-) mouse retina have also been observed, although the functional integrity of the visual pathway from retina to visual cortex is still unsettled. We have addressed this issue by characterizing the visual evoked potential (VEP) response of Npc1 (-/-) mice and determining if/how HPßCD administration influences the VEPs of both Npc1 (-/-) and Npc1 (+/+) mice. VEP elicited by a brief visual stimulus were recorded from the scalp overlying the visual cortex of adult (PN, postnatal days 60, 75, 85 and 100) Npc1 (+/+) and Npc1 (-/-) mice that had received repeated injections of either HPßCD or plain vehicle. The first injection was given at PN4 and was followed by a second one at PN7 and thereafter by weekly injections up to PN49. Cholesterol accumulation and myelin loss were finally assessed by filipin staining and myelin basic protein immunohistochemistry, respectively. We have found that the transmission of visual signals from retina to visual cortex is negatively influenced by the loss of Npc1 function. In fact, the VEP response of Npc1 (-/-) mice displayed a highly significant increase in the latency compared to that of Npc1 (+/+) mice. HPßCD administration fully rescued this defect and counteracted the cholesterol accumulation in retinal ganglion cells and dorsal lateral geniculate nucleus neurons, as well as the myelin loss in optic nerve fibers and axons projecting to the visual cortex observed in of Npc1 (-/-) mice. By contrast, HPßCD administration had no effect on the VEP response of Npc1 (+/+) mice, further strengthening the treatment efficacy. This study pinpoints the analysis of VEP response as a potentially accurate and non-invasive approach to assess neural activity and visual information processing in NPC1 patients, as well as for monitoring the progression of the disease and assessing the efficacy of potential therapies.

Twitter Demographics

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Mendeley readers

The data shown below were compiled from readership statistics for 27 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Germany 1 4%
Unknown 26 96%

Demographic breakdown

Readers by professional status Count As %
Researcher 8 30%
Student > Ph. D. Student 6 22%
Student > Bachelor 4 15%
Professor 1 4%
Other 1 4%
Other 2 7%
Unknown 5 19%
Readers by discipline Count As %
Agricultural and Biological Sciences 4 15%
Neuroscience 4 15%
Medicine and Dentistry 4 15%
Biochemistry, Genetics and Molecular Biology 3 11%
Veterinary Science and Veterinary Medicine 2 7%
Other 4 15%
Unknown 6 22%

Attention Score in Context

This research output has an Altmetric Attention Score of 1. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 16 October 2015.
All research outputs
#2,943,072
of 6,258,277 outputs
Outputs from Orphanet Journal of Rare Diseases
#633
of 957 outputs
Outputs of similar age
#103,286
of 194,524 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#27
of 43 outputs
Altmetric has tracked 6,258,277 research outputs across all sources so far. This one is in the 29th percentile – i.e., 29% of other outputs scored the same or lower than it.
So far Altmetric has tracked 957 research outputs from this source. They receive a mean Attention Score of 4.3. This one is in the 25th percentile – i.e., 25% of its peers scored the same or lower than it.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 194,524 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 36th percentile – i.e., 36% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 43 others from the same source and published within six weeks on either side of this one. This one is in the 27th percentile – i.e., 27% of its contemporaries scored the same or lower than it.