↓ Skip to main content

Comparing the Bbs10 complete knockout phenotype with a specific renal epithelial knockout one highlights the link between renal defects and systemic inactivation in mice

Overview of attention for article published in Cilia, August 2015
Altmetric Badge

About this Attention Score

  • Good Attention Score compared to outputs of the same age (68th percentile)
  • Good Attention Score compared to outputs of the same age and source (71st percentile)

Mentioned by

twitter
1 X user
patent
1 patent

Citations

dimensions_citation
29 Dimensions

Readers on

mendeley
27 Mendeley
You are seeing a free-to-access but limited selection of the activity Altmetric has collected about this research output. Click here to find out more.
Title
Comparing the Bbs10 complete knockout phenotype with a specific renal epithelial knockout one highlights the link between renal defects and systemic inactivation in mice
Published in
Cilia, August 2015
DOI 10.1186/s13630-015-0019-8
Pubmed ID
Authors

Noëlle Cognard, Maria J Scerbo, Cathy Obringer, Xiangxiang Yu, Fanny Costa, Elodie Haser, Dane Le, Corinne Stoetzel, Michel J Roux, Bruno Moulin, Hélène Dollfus, Vincent Marion

Abstract

Bardet-Biedl Syndrome (BBS) is a genetically heterogeneous ciliopathy with clinical cardinal features including retinal degeneration, obesity and renal dysfunction. To date, 20 BBS genes have been identified with BBS10 being a major BBS gene found to be mutated in almost 20 percent of all BBS patients worldwide. It codes for the BBS10 protein which forms part of a chaperone complex localized at the basal body of the primary cilium. Renal dysfunction in BBS patients is one of the major causes of morbidity in human patients and is associated initially with urinary concentration defects related to water reabsorption impairment in renal epithelial cells. The aim of this study was to study and compare the impact of a total Bbs10 inactivation (Bbs10 (-/-)) with that of a specific renal epithelial cells inactivation (Bbs10  (fl/fl) ; Cdh16-Cre (+/-)). We generated the Bbs10 (-/-) and Bbs10  (fl/fl) ; Cadh16-Cre (+/-) mouse model and characterized them. Bbs10 (-/-) mice developed obesity, retinal degeneration, structural defects in the glomeruli, polyuria associated with high circulating arginine vasopressin (AVP) concentrations, and vacuolated, yet ciliated, renal epithelial cells. On the other hand, the Bbs10  (fl/fl) ; Cadh16-Cre (+/-)mice displayed no detectable impairment. These data highlight the importance of a systemic Bbs10 inactivation to trigger averted renal dysfunction whereas a targeted absence of BBS10 in the renal epithelium is seemingly non-deleterious.

X Demographics

X Demographics

The data shown below were collected from the profile of 1 X user who shared this research output. Click here to find out more about how the information was compiled.
Mendeley readers

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 %
Unknown 27 100%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 8 30%
Researcher 6 22%
Student > Master 3 11%
Student > Doctoral Student 2 7%
Student > Postgraduate 2 7%
Other 4 15%
Unknown 2 7%
Readers by discipline Count As %
Biochemistry, Genetics and Molecular Biology 14 52%
Agricultural and Biological Sciences 6 22%
Medicine and Dentistry 5 19%
Nursing and Health Professions 1 4%
Pharmacology, Toxicology and Pharmaceutical Science 1 4%
Other 0 0%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 4. 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 26 January 2017.
All research outputs
#6,959,631
of 22,821,814 outputs
Outputs from Cilia
#32
of 91 outputs
Outputs of similar age
#81,253
of 264,395 outputs
Outputs of similar age from Cilia
#2
of 7 outputs
Altmetric has tracked 22,821,814 research outputs across all sources so far. This one has received more attention than most of these and is in the 68th percentile.
So far Altmetric has tracked 91 research outputs from this source. They receive a mean Attention Score of 4.9. This one has gotten more attention than average, scoring higher than 63% of its peers.
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 264,395 tracked outputs that were published within six weeks on either side of this one in any source. This one has gotten more attention than average, scoring higher than 68% of its contemporaries.
We're also able to compare this research output to 7 others from the same source and published within six weeks on either side of this one. This one has scored higher than 5 of them.