Building bridges, not walls: spinal cord regeneration in zebrafish [REVIEW]
ABSTRACT
Spinal cord injury is a devastating condition in which massive cell death and disruption of neural circuitry lead to long-term chronic functional impairment and paralysis. In mammals, spinal cord tissue has minimal capacity to regenerate after injury. In stark contrast, the regeneration of a completely transected spinal cord and accompanying reversal of paralysis in adult zebrafish is arguably one of the most spectacular biological phenomena in nature. Here, we review reports from the last decade that dissect the mechanisms of spinal cord regeneration in zebrafish. We highlight recent progress as well as areas req...
Source: DMM Disease Models and Mechanisms - May 26, 2020 Category: Biomedical Science Authors: Cigliola, V., Becker, C. J., Poss, K. D. Tags: Zebrafish as a Disease Model REVIEW Source Type: research
Activated pathogenic Th17 lymphocytes induce hypertension following high-fructose intake in Dahl salt-sensitive but not Dahl salt-resistant rats [RESEARCH ARTICLE]
ABSTRACT
High-salt intake and high-fructose intake are risk factors for hypertension via oxidative stress and inflammation. T helper (Th)17 lymphocytes play an important role in the development of hypertension. Here, we tested the hypothesis that activation of pathogenic Th17 lymphocytes induces hypertension after high-fructose intake in Dahl salt-sensitive (SS) but not Dahl salt-resistant (SR) rats. Eight-week-old male SS and SR rats were offered 20% fructose solution or tap water only for 4 weeks. Systolic blood pressure was measured by the tail-cuff method. T lymphocyte [Th17 and T regulatory (Treg)] profiling was ...
Source: DMM Disease Models and Mechanisms - May 26, 2020 Category: Biomedical Science Authors: Lee, E., Kim, N., Kang, J., Yoon, S., Lee, H.-A., Jung, H., Kim, S.-H., Kim, I. Tags: Rat as a Disease Model RESEARCH ARTICLE Source Type: research
First person - Anna Gray [FIRST PERSON]
ABSTRACT
First Person is a series of interviews with the first authors of a selection of papers published in Disease Models & Mechanisms (DMM), helping early-career researchers promote themselves alongside their papers. Anna Gray is first author on ‘ Deterioration of muscle force and contractile characteristics are early pathological events in spinal and bulbar muscular atrophy mice’, published in DMM. Anna conducted the research described in this article while a PhD student in Prof. Linda Greensmith's lab at the Department of Neuromuscular Disease, UCL Institute of Neurology, Queen Square, London, UK. She ...
Source: DMM Disease Models and Mechanisms - May 25, 2020 Category: Biomedical Science Tags: FIRST PERSON Source Type: research
First person - Kristin Ates [FIRST PERSON]
ABSTRACT
First Person is a series of interviews with the first authors of a selection of papers published in Disease Models & Mechanisms, helping early-career researchers promote themselves alongside their papers. Kristin Ates is first author on ‘ Deficiency in the endocytic adaptor proteins PHETA1/2 impairs renal and craniofacial development’, published in DMM. Kristin is an MD/PhD student in the lab of Y. Albert Pan at Augusta University, Augusta, GA, USA, investigating the underlying pathogenesis of endosomal and lysosomal diseases. (Source: DMM Disease Models and Mechanisms)
Source: DMM Disease Models and Mechanisms - May 25, 2020 Category: Biomedical Science Tags: FIRST PERSON Source Type: research
Modeling human epigenetic disorders in mice: Beckwith-Wiedemann syndrome and Silver-Russell syndrome [REVIEW]
ABSTRACT
Genomic imprinting, a phenomenon in which the two parental alleles are regulated differently, is observed in mammals, marsupials and a few other species, including seed-bearing plants. Dysregulation of genomic imprinting can cause developmental disorders such as Beckwith-Wiedemann syndrome (BWS) and Silver-Russell syndrome (SRS). In this Review, we discuss (1) how various (epi)genetic lesions lead to the dysregulation of clinically relevant imprinted loci, and (2) how such perturbations may contribute to the developmental defects in BWS and SRS. Given that the regulatory mechanisms of most imprinted clusters are w...
Source: DMM Disease Models and Mechanisms - May 25, 2020 Category: Biomedical Science Authors: Chang, S., Bartolomei, M. S. Tags: REVIEW Source Type: research
Deterioration of muscle force and contractile characteristics are early pathological events in spinal and bulbar muscular atrophy mice [RESEARCH ARTICLE]
This article has an associated First Person interview with the first author of the paper. (Source: DMM Disease Models and Mechanisms)
Source: DMM Disease Models and Mechanisms - May 25, 2020 Category: Biomedical Science Authors: Gray, A. L., Annan, L., Dick, J. R. T., La Spada, A. R., Hanna, M. G., Greensmith, L., Malik, B. Tags: Neuromuscular RESEARCH ARTICLE Source Type: research
Deficiency in the endocytic adaptor proteins PHETA1/2 impairs renal and craniofacial development [RESEARCH ARTICLE]
This article has an associated First Person interview with the first author of the paper. (Source: DMM Disease Models and Mechanisms)
Source: DMM Disease Models and Mechanisms - May 25, 2020 Category: Biomedical Science Authors: Ates, K. M., Wang, T., Moreland, T., Veeranan-Karmegam, R., Ma, M., Jeter, C., Anand, P., Wenzel, W., Kim, H.-G., Wolfe, L. A., Stephen, J., Adams, D. R., Markello, T., Tifft, C. J., Settlage, R., Gahl, W. A., Gonsalvez, G. B., Malicdan, M. C., Flanagan-S Tags: Zebrafish as a Disease Model RESEARCH ARTICLE Source Type: research
Temperature-sensitive spinal muscular atrophy-causing point mutations lead to SMN instability, locomotor defects and premature lethality in Drosophila [RESEARCH ARTICLE]
ABSTRACT
Spinal muscular atrophy (SMA) is the leading genetic cause of death in young children, arising from homozygous deletion or mutation of the survival motor neuron 1 (SMN1) gene. SMN protein expressed from a paralogous gene, SMN2, is the primary genetic modifier of SMA; small changes in overall SMN levels cause dramatic changes in disease severity. Thus, deeper insight into mechanisms that regulate SMN protein stability should lead to better therapeutic outcomes. Here, we show that SMA patient-derived missense mutations in the Drosophila SMN Tudor domain exhibit a pronounced temperature sensitivity that affects organ...
Source: DMM Disease Models and Mechanisms - May 21, 2020 Category: Biomedical Science Authors: Raimer, A. C., Singh, S. S., Edula, M. R., Paris-Davila, T., Vandadi, V., Spring, A. M., Matera, A. G. Tags: Neuromuscular, Drosophila as a Disease Model RESEARCH ARTICLE Source Type: research
Diverse dystonin gene mutations cause distinct patterns of Dst isoform deficiency and phenotypic heterogeneity in Dystonia musculorum mice [RESEARCH ARTICLE]
ABSTRACT
Loss-of-function mutations in dystonin (DST) can cause hereditary sensory and autonomic neuropathy type 6 (HSAN-VI) or epidermolysis bullosa simplex (EBS). Recently, DST-related diseases were recognized to be more complex than previously thought because a patient exhibited both neurological and skin manifestations, whereas others display only one or the other. A single DST locus produces at least three major DST isoforms: DST-a (neuronal isoform), DST-b (muscular isoform) and DST-e (epithelial isoform). Dystonia musculorum (dt) mice, which have mutations in Dst, were originally identified as spontaneous mutants di...
Source: DMM Disease Models and Mechanisms - May 20, 2020 Category: Biomedical Science Authors: Yoshioka, N., Kabata, Y., Kuriyama, M., Bizen, N., Zhou, L., Tran, D. M., Yano, M., Yoshiki, A., Ushiki, T., Sproule, T. J., Abe, R., Takebayashi, H. Tags: Neuromuscular RESEARCH ARTICLE Source Type: research
Genetic predisposition for increased red blood cell distribution width is an early risk factor for cardiovascular and renal comorbidities [RESEARCH ARTICLE]
ABSTRACT
Red blood cell distribution width (RDW) is a measurement of the variation in size and volume of red blood cells (RBCs). Increased RDW, indicating a high heterogeneity of RBCs, is prominently associated with a variety of illnesses, especially cardiovascular diseases. However, the significance of this association to the onset and progression of cardiovascular and renal diseases is unknown. We hypothesized that a genetic predisposition for increased RDW is an early risk factor for cardiovascular and renal comorbidities. Since there is no known animal model of increased RDW, we examined a CRISPR/Cas9 gene-edited rat m...
Source: DMM Disease Models and Mechanisms - May 16, 2020 Category: Biomedical Science Authors: Cheng, X., Mell, B., Alimadadi, A., Galla, S., McCarthy, C. G., Chakraborty, S., Basrur, V., Joe, B. Tags: RESEARCH ARTICLE Source Type: research
Correction: Vascular regression precedes motor neuron loss in the FUS (1-359) ALS mouse model [CORRECTION]
(Source: DMM Disease Models and Mechanisms)
Source: DMM Disease Models and Mechanisms - May 14, 2020 Category: Biomedical Science Authors: Crivello, M., Hogg, M. C., Jirström, E., Halang, L., Woods, I., Rayner, M., Coughlan, K. S., Lewandowski, S. A., Prehn, J. H. M. Tags: CORRECTION Source Type: research
The transcription factor Nurr1 is upregulated in amyotrophic lateral sclerosis patients and SOD1-G93A mice [RESEARCH ARTICLE]
ABSTRACT
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that affects both lower and upper motor neurons (MNs) in the central nervous system. ALS etiology is highly multifactorial and multifarious, and an effective treatment is still lacking. Neuroinflammation is a hallmark of ALS and could be targeted to develop new therapeutic approaches. Interestingly, the transcription factor Nurr1 has been demonstrated to have an important role in the inflammatory process in several neurological disorders, such as Parkinson's disease and multiple sclerosis. In the present paper, we demonstrate for the first time tha...
Source: DMM Disease Models and Mechanisms - May 14, 2020 Category: Biomedical Science Authors: Valsecchi, V., Boido, M., Montarolo, F., Guglielmotto, M., Perga, S., Martire, S., Cutrupi, S., Iannello, A., Gionchiglia, N., Signorino, E., Calvo, A., Fuda, G., Chio, A., Bertolotto, A., Vercelli, A. Tags: Neurodegenerative disorders, Neuromuscular RESEARCH ARTICLE Source Type: research
First person - Sofia de Oliveira [FIRST PERSON]
ABSTRACT
First Person is a series of interviews with the first authors of a selection of papers published in Disease Models & Mechanisms, helping early-career researchers promote themselves alongside their papers. Sofia de Oliveira is first author on ‘DnaJ-PKAc fusion induces liver inflammation in a zebrafish model of fibrolamellar carcinoma’, published in DMM. Sofia conducted the research described in this article while a postdoctoral fellow in Anna Huttenlocher's lab at the University of Wisconsin-Madison, Madison, WI, USA. She is now an assistant professor at Albert Einstein College of Medicine, Bronx, N...
Source: DMM Disease Models and Mechanisms - May 9, 2020 Category: Biomedical Science Tags: FIRST PERSON Source Type: research
First person - Sneh Harsh [FIRST PERSON]
ABSTRACT
First Person is a series of interviews with the first authors of a selection of papers published in Disease Models & Mechanisms, helping early-career researchers promote themselves alongside their papers. Sneh Harsh is first author on ‘Zika virus non-structural protein NS4A restricts eye growth in Drosophila through regulation of JAK/STAT signaling’, published in DMM. Sneh conducted the research described in this article while a postdoctoral researcher in Ioannis Eleftherianos's lab at The George Washington University, Washington, DC, USA. She is now a postdoctoral researcher in the lab of Erika Ba...
Source: DMM Disease Models and Mechanisms - April 29, 2020 Category: Biomedical Science Tags: FIRST PERSON Source Type: research
Membrane trafficking in health and disease [AT A GLANCE]
ABSTRACT
Membrane trafficking pathways are essential for the viability and growth of cells, and play a major role in the interaction of cells with their environment. In this At a Glance article and accompanying poster, we outline the major cellular trafficking pathways and discuss how defects in the function of the molecular machinery that mediates this transport lead to various diseases in humans. We also briefly discuss possible therapeutic approaches that may be used in the future treatment of trafficking-based disorders. (Source: DMM Disease Models and Mechanisms)
Source: DMM Disease Models and Mechanisms - April 29, 2020 Category: Biomedical Science Authors: Yarwood, R., Hellicar, J., Woodman, P. G., Lowe, M. Tags: AT A GLANCE Source Type: research
