For Publishers
DiscoveryMetadataPeer reviewHostingPublishing
For Researchers
JoinPublishReviewCollect
Register
Blog
About
Search
Advanced search
My ScienceOpen
Search
For Publishers
For Researchers
Blog
About
50
views
17
references
 Top references
cited by
2
    
0 reviews
 Review
0
comments
 Comment
0
recommends
+1 Recommend
0 collections
    4
    shares
      154
      similar
       All similar
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Myotonia Congenita-Associated Mutations in Chloride Channel-1 Affect Zebrafish Body Wave Swimming Kinematics

      research-article

      Read this article at

      ScienceOpenPublisherPMC
      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Myotonia congenita is a human muscle disorder caused by mutations in CLCN1, which encodes human chloride channel 1 (CLCN1). Zebrafish is becoming an increasingly useful model for human diseases, including muscle disorders. In this study, we generated transgenic zebrafish expressing, under the control of a muscle specific promoter, human CLCN1 carrying mutations that have been identified in human patients suffering from myotonia congenita. We developed video analytic tools that are able to provide precise quantitative measurements of movement abnormalities in order to analyse the effect of these CLCN1 mutations on adult transgenic zebrafish swimming. Two new parameters for body-wave kinematics of swimming reveal changes in body curvature and tail offset in transgenic zebrafish expressing the disease-associated CLCN1 mutants, presumably due to their effect on muscle function. The capability of the developed video analytic tool to distinguish wild-type from transgenic zebrafish could provide a useful asset to screen for compounds that reverse the disease phenotype, and may be applicable to other movement disorders besides myotonia congenita.

          Related collections

          Most cited references17

          • Record: found
          • Abstract: found
          • Article: not found

          The syntenic relationship of the zebrafish and human genomes.

          W. Barbazuk (2000)
          The zebrafish is an important vertebrate model for the mutational analysis of genes effecting developmental processes. Understanding the relationship between zebrafish genes and mutations with those of humans will require understanding the syntenic correspondence between the zebrafish and human genomes. High throughput gene and EST mapping projects in zebrafish are now facilitating this goal. Map positions for 523 zebrafish genes and ESTs with predicted human orthologs reveal extensive contiguous blocks of synteny between the zebrafish and human genomes. Eighty percent of genes and ESTs analyzed belong to conserved synteny groups (two or more genes linked in both zebrafish and human) and 56% of all genes analyzed fall in 118 homology segments (uninterrupted segments containing two or more contiguous genes or ESTs with conserved map order between the zebrafish and human genomes). This work now provides a syntenic relationship to the human genome for the majority of the zebrafish genome.
          Article 0 comments Cited 121 times – based on 0 reviews     Review now
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Locomotor repertoire of the larval zebrafish: swimming, turning and prey capture.

            S Budick, D O'Malley (2000)
            Larval zebrafish (Brachydanio rerio) are a popular model system because of their genetic attributes, transparency and relative simplicity. They have approximately 200 neurons that project from the brainstem into the spinal cord. Many of these neurons can be individually identified and laser-ablated in intact larvae. This should facilitate cellular-level characterization of the descending control of larval behavior patterns. Towards this end, we attempt to describe the range of locomotor behavior patterns exhibited by zebrafish larvae. Using high-speed digital imaging, a variety of swimming and turning behaviors were analyzed in 6- to 9-day-old larval fish. Swimming episodes appeared to fall into two categories, with the point of maximal bending of the larva's body occurring either near the mid-body (burst swims) or closer to the tail (slow swims). Burst swims also involved larger-amplitude bending, faster speeds and greater yaw than slow swims. Turning behaviors clearly fell into two distinct categories: fast, large-angle escape turns characteristic of escape responses, and much slower routine turns lacking the large counterbend that often accompanies escape turns. Prey-capture behaviors were also recorded. They were made up of simpler locomotor components that appeared to be similar to routine turns and slow swims. The different behaviors observed were analyzed with regard to possible underlying neural control systems. Our analysis suggests the existence of discrete sets of controlling neurons and helps to explain the need for the roughly 200 spinal-projecting nerve cells in the brainstem of the larval zebrafish.
            Article 0 comments Cited 120 times – based on 0 reviews     Review now
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Anxiolytic effects of nicotine in zebrafish.

              Zachary Bencan, D. Levin, D Cerutti (2007)
              Anxiolytic effects of nicotine have been documented in studies with rodents and humans. Understanding the neural basis of nicotine-induced anxiolysis can help both with developing better aids for smoking cessation as well as with the potential development of novel nicotinic ligands for treating anxiety. Complementary non-mammalian models may be useful for determining the molecular bases of nicotine effects on neurobehavioral function. The current project examined whether a zebrafish model of anxiety would be sensitive to nicotine. When zebrafish are placed in a novel environment, they dive to the bottom of the tank. In the wild, diving could help to escape predation. We tested the anxiolytic effect of nicotine on the novelty-elicited diving response and subsequent habituation. Zebrafish placed in a novel tank spent the majority of time at the bottom third of the tank during the first minute of a 5-min session and then show a gradual decrease in time spent at the tank bottom. Nicotine treatment at 100 mg/l for 3 min by immersion before testing caused a significant decrease in diving throughout the session, while 50 mg/l was effective during the first minute when the greatest bottom dwelling was seen in controls. Nicotine effects were reversed by the nicotinic antagonist mecamylamine given together with nicotine, but not when administered shortly before the test session after prior nicotine dosing. This implies that the effect of nicotine on diving was due to net stimulation at nicotinic receptors, an effect that is blocked by mecamylamine; and that once invoked, this effect is no longer dependent on continuing activation of nicotinic receptors. The effect of nicotine on diving did not seem to be the result of a general disorientation of the fish. The 100 mg/ml nicotine dose was shown in our earlier study to significantly improve spatial-discrimination learning in zebrafish. Nicotine-induced anxiolytic effects can be modeled in the zebrafish. This preparation will help in the investigation of the molecular bases of this effect.
              Article 0 comments Cited 120 times – based on 0 reviews     Review now
                Bookmark
                All references

                Author and article information

                Contributors
                David R. Borchelt: Role: Editor
                Journal
                Journal ID (nlm-ta): PLoS One
                Journal ID (iso-abbrev): PLoS ONE
                Journal ID (publisher-id): plos
                Journal ID (pmc): plosone
                Title: PLoS ONE
                Publisher: Public Library of Science (San Francisco, USA )
                ISSN (Electronic): 1932-6203
                Publication date Collection: 2014
                Publication date (Electronic): 1 August 2014
                Volume: 9
                Issue: 8
                Electronic Location Identifier: e103445
                Affiliations
                [1 ]Epithelial Cell Biology Laboratory, Institute of Molecular and Cell Biology, Agency for Science Technology and Research, Singapore, Singapore
                [2 ]Institute for Infocomm Research, Agency for Science Technology and Research, Singapore, Singapore
                [3 ]Department of Neurology and Deparment of Clinical Research, University of Bern, Bern, Switzerland
                [4 ]Department of Physiology, National University of Singapore, Singapore, Singapore
                [5 ]Singapore Eye Research Institute, Singapore, Singapore
                University of Florida, United States of America
                Author notes

                ¶ WC and JT are co-first authors on this work.

                Competing Interests: The authors have declared that no competing interests exist.

                Conceived and designed the experiments: WC JT JMB WH HLE. Performed the experiments: WC JT. Analyzed the data: WC JT JMB WH HLE. Contributed reagents/materials/analysis tools: WC JT JMB WH HLE. Contributed to the writing of the manuscript: WC JT JMB WH HLE.

                [¤]

                Current address: ZWEEC Analytics Pte Ltd, Singapore, Singapore

                Article
                Publisher ID: PONE-D-14-13698
                DOI: 10.1371/journal.pone.0103445
                PMC ID: 4118878
                PubMed ID: 25083883
                SO-VID: a0a3d2bd-0975-4d3e-99a4-d9bf54f26f5e
                Copyright statement: Copyright @ 2014
                License:

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                Date received : 27 March 2014
                Date accepted : 30 June 2014
                Page count
                Pages: 10
                Funding
                This work was funded by grants 10/03/EG/05/02 and 1031C004 from the Joint Council Office of the Agency for Science Technology and Research (A*STAR), Singapore. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
                Categories
                Subject: Research Article
                Subject: Biology and Life Sciences
                Subject: Anatomy
                Subject: Musculoskeletal System
                Subject: Muscles
                Subject: Muscle Fibers
                Subject: Biochemistry
                Subject: Proteins
                Subject: Ion Channels
                Subject: Organisms
                Subject: Animals
                Subject: Vertebrates
                Subject: Fishes
                Subject: Osteichthyes
                Subject: Zebrafish
                Subject: Medicine and Health Sciences
                Subject: Neurology
                Subject: Neurodegenerative Diseases
                Subject: Movement Disorders
                Subject: Research and Analysis Methods
                Subject: Model Organisms
                Subject: Animal Models
                Custom metadata
                Data Availability The authors confirm that all data underlying the findings are fully available without restriction. The source code with and example video are provided as Supporting Information files.

                ScienceOpen disciplines: Uncategorized
                Data availability:
                ScienceOpen disciplines: Uncategorized

                Comments

                Comment on this article

                scite_

                Similar content154

                See all similar

                Cited by2

                See all cited by

                Most referenced authors116

                See all reference authors