Automated Author ProfileBredford Kerr
Bredford Kerr
Current S-Index
Sum of Dataset Indices for all datasets
Average Dataset Index per Dataset
Average Dataset Index per dataset
Total Datasets
Total datasets for this author
Average FAIR Score
Average FAIR Score per dataset
Total Citations
Total citations to the author's datasets
Total Mentions
Total mentions of the author's datasets
S-Index Interpretation
The S-Index (Sharing Index) is a comprehensive metric that represents the cumulative impact of all your datasets. It is calculated as the sum of Dataset Index scores across all your claimed datasets.
What it means:
- A higher S-index indicates greater overall impact of your datasets relative to typical datasets in their fields of research
- The S-Index grows as you add more datasets or as existing datasets gain more citations and mentions
- It provides a single number to track your research data impact over time
Current S-Index: 5.3 (sum of 11 datasets Dataset Index scores)
More information here.
S-Index Over Time
Cumulative Citations Over Time
Cumulative Mentions Over Time
Datasets
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by loss of motoneurons and compromised proteostasis. Dysfunction of the endoplasmic reticulum (ER) has been identified as a transversal pathogenic mechanism associated to motoneurons vulnerability in ALS. Protein disulfide isomerases (PDIs) are key enzymes catalyzing protein folding at the ER that are altered in the disease, involving both biochemical and genetic perturbations. We previously identified mutations in the gene encoding PDIA3 (also known as Grp58 or ERp57) in ALS cases, which were associated with altered neurite outgrowth in cell culture and abnormal motoneuron connectivity in zebrafish. Here we report the generation of transgenic mice expressing the ALS-associated PDIA3Q481K variant. Moderate PDIA3Q481K overexpression resulted in altered motor capacity accompanied by decreased motoneurons number and induction of ER stress in the spinal cord. The adverse effects of PDIA3Q481K were associated with altered electromyogram without evident morphological changes in neuromuscular junctions. Our results suggest that the PDIA3Q481K variant is pathogenic and its overexpression in mice recapitulate some ALS features, further supporting the concept that altered proteostasis due to PDI dysfunction constitute a risk factor to develop the disease.
Authors
- Hetz, Claudio ;
- Medinas, Danilo B. ;
- Henriquez, Juan Pablo ;
- Woehlbier, Ute ;
- Bredford Kerr ;
- Diaz, Guillermo ;
- Zuleta, Amparo ;
- Mansilla-Jaramillo, Matías ;
- Rozas, Pablo ;
- Mella, Jessica ;
- Ojeda, Jorge ;
- Perez, Viviana ;
- Ojeda, Patricia ;
- Martinez-Traub1, Francisca ;
- Sepulveda, Martin
:unav
Authors
- Hetz, Claudio ;
- Medinas, Danilo B. ;
- Henriquez, Juan Pablo ;
- Woehlbier, Ute ;
- Bredford Kerr ;
- Diaz, Guillermo ;
- Zuleta, Amparo ;
- Mansilla-Jaramillo, Matías ;
- Rozas, Pablo ;
- Mella, Jessica ;
- Ojeda, Jorge ;
- Perez, Viviana ;
- Ojeda, Patricia ;
- Martinez-Traub1, Francisca ;
- Sepulveda, Martin
:unav
Authors
- Hetz, Claudio ;
- Medinas, Danilo B. ;
- Henriquez, Juan Pablo ;
- Woehlbier, Ute ;
- Bredford Kerr ;
- Diaz, Guillermo ;
- Zuleta, Amparo ;
- Mansilla-Jaramillo, Matías ;
- Rozas, Pablo ;
- Mella, Jessica ;
- Ojeda, Jorge ;
- Perez, Viviana ;
- Ojeda, Patricia ;
- Martinez-Traub1, Francisca ;
- Sepulveda, Martin
:unav
Authors
- Hetz, Claudio ;
- Medinas, Danilo B. ;
- Henriquez, Juan Pablo ;
- Woehlbier, Ute ;
- Bredford Kerr ;
- Diaz, Guillermo ;
- Zuleta, Amparo ;
- Mansilla-Jaramillo, Matías ;
- Rozas, Pablo ;
- Mella, Jessica ;
- Ojeda, Jorge ;
- Perez, Viviana ;
- Ojeda, Patricia ;
- Martinez-Traub1, Francisca ;
- Sepulveda, Martin
:unav
Authors
- Hetz, Claudio ;
- Medinas, Danilo B. ;
- Henriquez, Juan Pablo ;
- Woehlbier, Ute ;
- Bredford Kerr ;
- Diaz, Guillermo ;
- Zuleta, Amparo ;
- Mansilla-Jaramillo, Matías ;
- Rozas, Pablo ;
- Mella, Jessica ;
- Ojeda, Jorge ;
- Perez, Viviana ;
- Ojeda, Patricia ;
- Martinez-Traub1, Francisca ;
- Sepulveda, Martin
:unav
Authors
- Hetz, Claudio ;
- Medinas, Danilo B. ;
- Henriquez, Juan Pablo ;
- Woehlbier, Ute ;
- Bredford Kerr ;
- Diaz, Guillermo ;
- Zuleta, Amparo ;
- Mansilla-Jaramillo, Matías ;
- Rozas, Pablo ;
- Mella, Jessica ;
- Ojeda, Jorge ;
- Perez, Viviana ;
- Ojeda, Patricia ;
- Martinez-Traub1, Francisca ;
- Sepulveda, Martin
:unav
Authors
- Hetz, Claudio ;
- Medinas, Danilo B. ;
- Henriquez, Juan Pablo ;
- Woehlbier, Ute ;
- Bredford Kerr ;
- Diaz, Guillermo ;
- Zuleta, Amparo ;
- Mansilla-Jaramillo, Matías ;
- Rozas, Pablo ;
- Mella, Jessica ;
- Ojeda, Jorge ;
- Perez, Viviana ;
- Ojeda, Patricia ;
- Martinez-Traub1, Francisca ;
- Sepulveda, Martin
:unav
Authors
- Hetz, Claudio ;
- Medinas, Danilo B. ;
- Henriquez, Juan Pablo ;
- Woehlbier, Ute ;
- Bredford Kerr ;
- Diaz, Guillermo ;
- Zuleta, Amparo ;
- Mansilla-Jaramillo, Matías ;
- Rozas, Pablo ;
- Mella, Jessica ;
- Ojeda, Jorge ;
- Perez, Viviana ;
- Ojeda, Patricia ;
- Martinez-Traub1, Francisca ;
- Sepulveda, Martin
:unav
Authors
- Hetz, Claudio ;
- Medinas, Danilo B. ;
- Henriquez, Juan Pablo ;
- Woehlbier, Ute ;
- Bredford Kerr ;
- Diaz, Guillermo ;
- Zuleta, Amparo ;
- Mansilla-Jaramillo, Matías ;
- Rozas, Pablo ;
- Mella, Jessica ;
- Ojeda, Jorge ;
- Perez, Viviana ;
- Ojeda, Patricia ;
- Martinez-Traub1, Francisca ;
- Sepulveda, Martin
:unav
Authors
- Hetz, Claudio ;
- Medinas, Danilo B. ;
- Henriquez, Juan Pablo ;
- Woehlbier, Ute ;
- Bredford Kerr ;
- Diaz, Guillermo ;
- Zuleta, Amparo ;
- Mansilla-Jaramillo, Matías ;
- Rozas, Pablo ;
- Mella, Jessica ;
- Ojeda, Jorge ;
- Perez, Viviana ;
- Ojeda, Patricia ;
- Martinez-Traub1, Francisca ;
- Sepulveda, Martin