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Friedreich's ataxia (FRDA), the most common inherited ataxia, is caused by recessive mutations that reduce the levels of frataxin (FXN), a mitochondrial iron binding protein. We developed an inducible mouse model of Fxn deficiency that enabled us to control the onset and progression of disease phenotypes by the modulation of Fxn levels. Systemic knockdown of Fxn in adult mice led to multiple phenotypes paralleling those observed in human patients across multiple organ systems. By reversing knockdown after clinical features appear, we were able to determine to what extent observed phenotypes represent reversible cellular dysfunction. Remarkably, upon restoration of near wild-type FXN levels, we observed significant recovery of function, associated pathology and transcriptomic dysregulation even after substantial motor dysfunction and pathology were observed. This model will be of broad utility in therapeutic development and in refining our understanding of the relative contribution of reversible cellular dysfunction at different stages in disease.
Pubmed ID: 29257745
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View all literature mentionsCommercial instrument and chemical vendor. Developer and manufacturer of specialized technological products for life science research and clinical diagnostics markets.
View all literature mentionsPrivately held company that develops and produces antibodies, ELISA kits, ChIP kits, proteomic kits, and other related reagents used to study cell signaling pathways that impact human health.
View all literature mentionsSoftware tool to enable biologists without training in computer vision or programming to quantitatively measure phenotypes from thousands of images automatically. It counts cells and also measures the size, shape, intensity and texture of every cell (and every labeled subcellular compartment) in every image. It was designed for high throughput screening but can perform automated image analysis for images from time-lapse movies and low-throughput experiments. CellProfiler has an increasing number of algorithms to identify and measure properties of neuronal cell types.
View all literature mentionslaboratory mouse with name 129/SvEv from MGI.
View all literature mentionsThis polyclonal targets FXN
View all literature mentionsThis unknown targets Chicken IgG (H+L)
View all literature mentionsThis monoclonal targets Cleaved Caspase-8 (Asp387)
View all literature mentionsThis polyclonal targets Ferritin Light Chain
View all literature mentionsThis monoclonal targets Akt (pan) (C67E7) Rabbit mAb
View all literature mentionsThis polyclonal targets IgG (H+L)
View all literature mentionsThis polyclonal targets 4 Hydroxynonenal antibody
View all literature mentionsThis polyclonal secondary targets IgG (H+L)
View all literature mentionsThis monoclonal targets 3-Nitrotyrosine antibody [39B6]
View all literature mentionsThis monoclonal targets Pdk1
View all literature mentionsThis monoclonal targets Map1lc3b
View all literature mentionsThis polyclonal targets SLC40A1
View all literature mentionsThis polyclonal secondary targets IgG (H+L)
View all literature mentionsThis polyclonal secondary targets IgG (H+L) Poly-HRP
View all literature mentionsThis monoclonal targets β-Actin
View all literature mentionsThis polyclonal targets Rabbit NeuN (polyclonal)
View all literature mentionsSoftware package for the analysis of gene expression microarray data, especially the use of linear models for analyzing designed experiments and the assessment of differential expression.
View all literature mentionsOpen source Java based image processing software program designed for scientific multidimensional images. ImageJ has been transformed to ImageJ2 application to improve data engine to be sufficient to analyze modern datasets.
View all literature mentionsOpen source Java based image processing software program designed for scientific multidimensional images. ImageJ has been transformed to ImageJ2 application to improve data engine to be sufficient to analyze modern datasets.
View all literature mentionsPubMatrix is a web-based tool that allows simple text based mining of the NCBI literature search service PubMed using any two lists of keywords terms, resulting in a frequency matrix of term co-occurrence. PubMatrix is a simple way to rapidly and systematically compare any list of terms against any other list of terms in PubMed. It reports back the frequency of co-occurrence between all pairwise comparisons between the two lists as a matrix table. Lists of terms can be anything; gene names, diseases, gene functions, authors, etc. The user can then quickly sort or browse the frequency matrix table to do individual searches independently. This allows the user to build up tables of word relationships in PubMed in the context of your experiments or your scientific interests. This is useful for analyzing combinatorial datasets, as found with multiplex experimental systems, such as cDNA microarrays, genomic, proteomic, or other multiplex comparisons. The PubMatrix database is an archive of previous searches on many topics. Sponsors: PubMatrix is supported by the National Institutes of Health.
View all literature mentionsSoftware R package for weighted correlation network analysis. WGCNA is also available as point-and-click application. Unfortunately this application is not maintained anymore. It is known to have compatibility problems with R-2.8.x and newer, and the methods it implements are not all state of the art.
View all literature mentionsThis monoclonal targets Cleaved Caspase-8 (Asp387)
View all literature mentionsSoftware package for the analysis of gene expression microarray data, especially the use of linear models for analyzing designed experiments and the assessment of differential expression.
View all literature mentionsPubMatrix is a web-based tool that allows simple text based mining of the NCBI literature search service PubMed using any two lists of keywords terms, resulting in a frequency matrix of term co-occurrence. PubMatrix is a simple way to rapidly and systematically compare any list of terms against any other list of terms in PubMed. It reports back the frequency of co-occurrence between all pairwise comparisons between the two lists as a matrix table. Lists of terms can be anything; gene names, diseases, gene functions, authors, etc. The user can then quickly sort or browse the frequency matrix table to do individual searches independently. This allows the user to build up tables of word relationships in PubMed in the context of your experiments or your scientific interests. This is useful for analyzing combinatorial datasets, as found with multiplex experimental systems, such as cDNA microarrays, genomic, proteomic, or other multiplex comparisons. The PubMatrix database is an archive of previous searches on many topics. Sponsors: PubMatrix is supported by the National Institutes of Health.
View all literature mentionsCell line Neuro-2a is a Cancer cell line with a species of origin Mus musculus
View all literature mentionsSlide image modification software that allows the user to adjust the magnification, compare slides, pan and zoom, annotate specific areas, and perform image analysis of digital slides. Users can create macros and algorithms to automate analysis and create plots, respectively.
View all literature mentionsThis polyclonal targets FXN
View all literature mentionsBioinformatics resource system including web server and web service for functional annotation and enrichment analyses of gene lists. Consists of comprehensive knowledgebase and set of functional analysis tools. Includes gene centered database integrating heterogeneous gene annotation resources to facilitate high throughput gene functional analysis.
View all literature mentionsThis polyclonal targets IgG (H+L)
View all literature mentionsThis polyclonal targets FXN
View all literature mentionsA single point video tracking system. It uses the center of the mass of the animal and tracks it over the course of the experiment to determine location, distance traveled, velocity etc. Examples of applications include: Open Field, Novel Object Recognition, Maze Analysis, Morris Water Maze, Barnes Maze, Place Preference, etc.
View all literature mentionsSoftware for viewing and analyzing medical DICOM images. The software suite includes a viewer, a report generating feature, and a DICOM network connection.
View all literature mentionsWeb application to search nucleotide databases using a nucleotide query. Algorithms: blastn, megablast, discontiguous megablast.
View all literature mentionsSoftware that acquires, displays and analyzes physiological signals. It covers cardiovascular, respiratory and nervous system research areas and technologies from in vitro action potentials to live in vivo physiological signals monitoring with video and implantable telemetry.
View all literature mentionsThis polyclonal targets IgG (H+L)
View all literature mentionsA single point video tracking system. It uses the center of the mass of the animal and tracks it over the course of the experiment to determine location, distance traveled, velocity etc. Examples of applications include: Open Field, Novel Object Recognition, Maze Analysis, Morris Water Maze, Barnes Maze, Place Preference, etc.
View all literature mentionsA single point video tracking system. It uses the center of the mass of the animal and tracks it over the course of the experiment to determine location, distance traveled, velocity etc. Examples of applications include: Open Field, Novel Object Recognition, Maze Analysis, Morris Water Maze, Barnes Maze, Place Preference, etc.
View all literature mentionsSoftware for viewing and analyzing medical DICOM images. The software suite includes a viewer, a report generating feature, and a DICOM network connection.
View all literature mentionsThis polyclonal targets Ferritin Light Chain
View all literature mentionsThis polyclonal secondary targets IgG (H+L)
View all literature mentionsThis monoclonal targets Cleaved Caspase-8 (Asp387)
View all literature mentionsBioinformatics resource system including web server and web service for functional annotation and enrichment analyses of gene lists. Consists of comprehensive knowledgebase and set of functional analysis tools. Includes gene centered database integrating heterogeneous gene annotation resources to facilitate high throughput gene functional analysis.
View all literature mentionsThis polyclonal secondary targets IgG (H+L)
View all literature mentionsThis polyclonal secondary targets IgG (H+L) Poly-HRP
View all literature mentionsOpen source Java based image processing software program designed for scientific multidimensional images. ImageJ has been transformed to ImageJ2 application to improve data engine to be sufficient to analyze modern datasets.
View all literature mentionsMus musculus with name C57BL/6J from IMSR.
View all literature mentionsOpen source Java based image processing software program designed for scientific multidimensional images. ImageJ has been transformed to ImageJ2 application to improve data engine to be sufficient to analyze modern datasets.
View all literature mentionsThis monoclonal targets Map1lc3b
View all literature mentionsMus musculus with name C57BL/6J from IMSR.
View all literature mentionsCell line Neuro-2a is a Cancer cell line with a species of origin Mus musculus
View all literature mentionsThis polyclonal targets Rabbit NeuN (polyclonal)
View all literature mentionsThis polyclonal targets Rabbit NeuN (polyclonal)
View all literature mentionsOpen source Java based image processing software program designed for scientific multidimensional images. ImageJ has been transformed to ImageJ2 application to improve data engine to be sufficient to analyze modern datasets.
View all literature mentionsSoftware tool to enable biologists without training in computer vision or programming to quantitatively measure phenotypes from thousands of images automatically. It counts cells and also measures the size, shape, intensity and texture of every cell (and every labeled subcellular compartment) in every image. It was designed for high throughput screening but can perform automated image analysis for images from time-lapse movies and low-throughput experiments. CellProfiler has an increasing number of algorithms to identify and measure properties of neuronal cell types.
View all literature mentionsThis polyclonal secondary targets IgG (H+L) Poly-HRP
View all literature mentionsWeb application to search nucleotide databases using a nucleotide query. Algorithms: blastn, megablast, discontiguous megablast.
View all literature mentionsSoftware tool to enable biologists without training in computer vision or programming to quantitatively measure phenotypes from thousands of images automatically. It counts cells and also measures the size, shape, intensity and texture of every cell (and every labeled subcellular compartment) in every image. It was designed for high throughput screening but can perform automated image analysis for images from time-lapse movies and low-throughput experiments. CellProfiler has an increasing number of algorithms to identify and measure properties of neuronal cell types.
View all literature mentionsSlide image modification software that allows the user to adjust the magnification, compare slides, pan and zoom, annotate specific areas, and perform image analysis of digital slides. Users can create macros and algorithms to automate analysis and create plots, respectively.
View all literature mentionsThis monoclonal targets Map1lc3b
View all literature mentionsThis unknown targets Chicken IgG (H+L)
View all literature mentionsSoftware R package for weighted correlation network analysis. WGCNA is also available as point-and-click application. Unfortunately this application is not maintained anymore. It is known to have compatibility problems with R-2.8.x and newer, and the methods it implements are not all state of the art.
View all literature mentionsThis polyclonal secondary targets IgG (H+L)
View all literature mentionsSlide image modification software that allows the user to adjust the magnification, compare slides, pan and zoom, annotate specific areas, and perform image analysis of digital slides. Users can create macros and algorithms to automate analysis and create plots, respectively.
View all literature mentionsThis polyclonal targets Ferritin Light Chain
View all literature mentionsThis monoclonal targets 3-Nitrotyrosine antibody [39B6]
View all literature mentionsThis monoclonal targets Pdk1
View all literature mentionsA single point video tracking system. It uses the center of the mass of the animal and tracks it over the course of the experiment to determine location, distance traveled, velocity etc. Examples of applications include: Open Field, Novel Object Recognition, Maze Analysis, Morris Water Maze, Barnes Maze, Place Preference, etc.
View all literature mentionsThis monoclonal targets Akt (pan) (C67E7) Rabbit mAb
View all literature mentionsSoftware R package for weighted correlation network analysis. WGCNA is also available as point-and-click application. Unfortunately this application is not maintained anymore. It is known to have compatibility problems with R-2.8.x and newer, and the methods it implements are not all state of the art.
View all literature mentionsSlide image modification software that allows the user to adjust the magnification, compare slides, pan and zoom, annotate specific areas, and perform image analysis of digital slides. Users can create macros and algorithms to automate analysis and create plots, respectively.
View all literature mentionsBioinformatics resource system including web server and web service for functional annotation and enrichment analyses of gene lists. Consists of comprehensive knowledgebase and set of functional analysis tools. Includes gene centered database integrating heterogeneous gene annotation resources to facilitate high throughput gene functional analysis.
View all literature mentionsThis polyclonal targets 4 Hydroxynonenal antibody
View all literature mentionsThis monoclonal targets β-Actin
View all literature mentionsCell line Neuro-2a is a Cancer cell line with a species of origin Mus musculus
View all literature mentionsSoftware tool to enable biologists without training in computer vision or programming to quantitatively measure phenotypes from thousands of images automatically. It counts cells and also measures the size, shape, intensity and texture of every cell (and every labeled subcellular compartment) in every image. It was designed for high throughput screening but can perform automated image analysis for images from time-lapse movies and low-throughput experiments. CellProfiler has an increasing number of algorithms to identify and measure properties of neuronal cell types.
View all literature mentionsThis unknown targets Chicken IgG (H+L)
View all literature mentionsThis polyclonal targets SLC40A1
View all literature mentionsPubMatrix is a web-based tool that allows simple text based mining of the NCBI literature search service PubMed using any two lists of keywords terms, resulting in a frequency matrix of term co-occurrence. PubMatrix is a simple way to rapidly and systematically compare any list of terms against any other list of terms in PubMed. It reports back the frequency of co-occurrence between all pairwise comparisons between the two lists as a matrix table. Lists of terms can be anything; gene names, diseases, gene functions, authors, etc. The user can then quickly sort or browse the frequency matrix table to do individual searches independently. This allows the user to build up tables of word relationships in PubMed in the context of your experiments or your scientific interests. This is useful for analyzing combinatorial datasets, as found with multiplex experimental systems, such as cDNA microarrays, genomic, proteomic, or other multiplex comparisons. The PubMatrix database is an archive of previous searches on many topics. Sponsors: PubMatrix is supported by the National Institutes of Health.
View all literature mentionsSoftware package for the analysis of gene expression microarray data, especially the use of linear models for analyzing designed experiments and the assessment of differential expression.
View all literature mentionsThis polyclonal targets SLC40A1
View all literature mentionsSoftware that acquires, displays and analyzes physiological signals. It covers cardiovascular, respiratory and nervous system research areas and technologies from in vitro action potentials to live in vivo physiological signals monitoring with video and implantable telemetry.
View all literature mentionsThis monoclonal targets β-Actin
View all literature mentions