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Zebrafish disease modelling

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https://www.readbyqxmd.com/read/28449103/novel-degenerative-and-developmental-defects-in-a-zebrafish-model-of-mucolipidosis-type-iv
#1
Huiqing Li, Wuhong Pei, Sivia Vergarajauregui, Patricia M Zerfas, Nina Raben, Shawn M Burgess, Rosa Puertollano
Mucolipidosis type IV (MLIV) is a lysosomal storage disease characterized by neurologic and ophthalmologic abnormalities. There is currently no effective treatment. MLIV is caused by mutations in MCOLN1, a lysosomal cation channel from the transient receptor potential (TRP) family. In this study we used genome editing to knockout the two mcoln1 genes present in Dario rerio (zebrafish). Our model successfully reproduced the retinal and neuromuscular defects observed in MLIV patients, indicating that this model is suitable for studying the disease pathogenesis...
April 25, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28444311/expression-of-als-ftd-linked-mutant-ccnf-in-zebrafish-leads-to-increased-cell-death-in-the-spinal-cord-and-an-aberrant-motor-phenotype
#2
Alison L Hogan, Emily K Don, Stephanie L Rayner, Albert Lee, Angela S Laird, Maxinne Watchon, Claire Winnick, Ingrid S Tarr, Marco Morsch, Jennifer A Fifita, Serene Gwee, Isabel Formella, Elinor Hortle, Kristy Yuan, Mark P Molloy, Kelly L Williams, Garth A Nicholson, Roger S Chung, Ian P Blair, Nicholas J Cole
Amyotrophic lateral sclerosis (ALS) is a rapidly progressive, fatal neurodegenerative disease characterised by the death of upper and lower motor neurons. Approximately 10% of cases have a known family history of ALS and disease-linked mutations in multiple genes have been identified. ALS-linked mutations in CCNF were recently reported, however the pathogenic mechanisms associated with these mutations are yet to be established. To investigate possible disease mechanisms, we developed in vitro and in vivo models based on an ALS-linked missense mutation in CCNF...
April 21, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28439518/diabetes-adult-neurogenesis-and-brain-remodeling-new-insights-from-rodent-and-zebrafish-models
#3
REVIEW
Anne-Claire Dorsemans, David Couret, Anaïs Hoarau, Olivier Meilhac, Christian Lefebvre d'Hellencourt, Nicolas Diotel
The prevalence of diabetes rapidly increased during the last decades in association with important changes in lifestyle. Diabetes and hyperglycemia are well-known for inducing deleterious effects on physiologic processes, increasing for instance cardiovascular diseases, nephropathy, retinopathy and foot ulceration. Interestingly, diabetes also impairs brain morphology and functions such as (1) decreased neurogenesis (proliferation, differentiation and cell survival), (2) decreased brain volumes, (3) increased blood-brain barrier leakage, (4) increased cognitive impairments, as well as (5) increased stroke incidence and worse neurologic outcomes following stroke...
2017: Neurogenesis (Austin, Tex.)
https://www.readbyqxmd.com/read/28431223/pink1-based-screen-shines-light-on-autophagy-enhancers-for-parkinson-s-disease
#4
Dominik Haddad, Ken Nakamura
In this issue of Cell Chemical Biology, Zhang et al. (2017) report a zebrafish model of Parkinson's disease (PD), incorporating the PD-protein PINK1 and rotenone, a toxin linked to PD. Using it as a drug-screening platform, they identify trifluoperazine and other piperazine phenothiazines as protective compounds that enhance autophagy independent of PINK1.
April 20, 2017: Cell Chemical Biology
https://www.readbyqxmd.com/read/28426725/dynamic-regulation-of-pin1-expression-and-function-during-zebrafish-development
#5
Maria Solange Ibarra, Carla Borini Etichetti, Carolina Di Benedetto, Germán L Rosano, Ezequiel Margarit, Giannino Del Sal, Marina Mione, Javier Girardini
The prolyl isomerase Pin1 plays a key role in the modulation of proline-directed phosphorylation signaling by inducing local conformational changes in phosphorylated protein substrates. Extensive studies showed different roles for Pin1 in physiological processes and pathological conditions such as cancer and neurodegenerative diseases. However, there are still several unanswered questions regarding its biological role. Notably, despite evidences from cultured cells showing that Pin1 expression and activity may be regulated by different mechanisms, little is known on their relevance in vivo...
2017: PloS One
https://www.readbyqxmd.com/read/28426667/bioenergetic-status-modulates-motor-neuron-vulnerability-and-pathogenesis-in-a-zebrafish-model-of-spinal-muscular-atrophy
#6
Penelope J Boyd, Wen-Yo Tu, Hannah K Shorrock, Ewout J N Groen, Roderick N Carter, Rachael A Powis, Sophie R Thomson, Derek Thomson, Laura C Graham, Anna A L Motyl, Thomas M Wishart, J Robin Highley, Nicholas M Morton, Thomas Becker, Catherina G Becker, Paul R Heath, Thomas H Gillingwater
Degeneration and loss of lower motor neurons is the major pathological hallmark of spinal muscular atrophy (SMA), resulting from low levels of ubiquitously-expressed survival motor neuron (SMN) protein. One remarkable, yet unresolved, feature of SMA is that not all motor neurons are equally affected, with some populations displaying a robust resistance to the disease. Here, we demonstrate that selective vulnerability of distinct motor neuron pools arises from fundamental modifications to their basal molecular profiles...
April 20, 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28423345/homocysteine-induced-peripheral-microcirculation-dysfunction-in-zebrafish-and-its-attenuation-by-l-arginine
#7
Sang Joon Lee, Sung Ho Park, Jinhyuk Fred Chung, Woorak Choi, Hyung Kyu Huh
Elevated blood homocysteine (Hcy) level is frequently observed in aged individuals and those with age-related vascular diseases. However, its effect on peripheral microcirculation is still not fully understood. Using in vivo zebrafish model, the degree of Hcy-induced peripheral microcirculation dysfunction is assessed in this study with a proposed dimensionless velocity parameter V¯CV/V¯PCV, where V¯CV and V¯PCV represent the peripheral microcirculation perfusion and the systemic perfusion levels, respectively...
April 4, 2017: Oncotarget
https://www.readbyqxmd.com/read/28422363/the-occhiolino-occ-mutant-zebrafish-a-model-for-development-of-the-optical-function-in-the-biological-lens
#8
Masamoto Aose, Tor H Linbo, Owen Lawrence, Tadashi Senoo, David W Raible, John I Clark
BACKGROUND: Zebrafish visual function depends on quality optics. An F3 screen for developmental mutations in the zebrafish nervous system was conducted in wild-type (wt) AB zebrafish exposed to 3 mM of N-ethyl-N-nitrosourea (ENU). RESULTS: Mutant offspring, identified in an F3 screen, were characterized by a small pupil, resulting from retinal hypertrophy or hyperplasia and a small lens. Deficits in visual function made feeding difficult after hatching at approximately 5-6 dpf (days post fertilization)...
April 19, 2017: Developmental Dynamics: An Official Publication of the American Association of Anatomists
https://www.readbyqxmd.com/read/28422304/antibiotic-treatment-of-zebrafish-mycobacteriosis-tolerance-and-efficacy-of-treatments-with-tigecycline-and-clarithromycin
#9
C T Chang, K M Doerr, C M Whipps
Zebrafish (Danio rerio) are a popular model organism used in a growing number of research fields. Maintaining healthy, disease-free laboratory fish is important for the integrity of many of these studies. Mycobacteriosis is a chronic bacterial infection caused by several Mycobacterium spp. and is the second most common disease found in laboratory zebrafish. Current mycobacteriosis control measures recommend the removal of infected fish and in severe outbreaks, depopulation. These measures can be effective, but less disruptive measures should be assessed for controlling mycobacteriosis, particularly when valuable and rare lines of fish are affected...
April 19, 2017: Journal of Fish Diseases
https://www.readbyqxmd.com/read/28421165/the-diverse-cellular-and-animal-models-to-decipher-the-physiopathological-traits-of-mycobacterium-abscessus-infection
#10
REVIEW
Audrey Bernut, Jean-Louis Herrmann, Diane Ordway, Laurent Kremer
Mycobacterium abscessus represents an important respiratory pathogen among the rapidly-growing non-tuberculous mycobacteria. Infections caused by M. abscessus are increasingly found in cystic fibrosis (CF) patients and are often refractory to antibiotic therapy. The underlying immunopathological mechanisms of pathogenesis remain largely unknown. A major reason for the poor advances in M. abscessus research has been a lack of adequate models to study the acute and chronic stages of the disease leading to delayed progress of evaluation of therapeutic efficacy of potentially active antibiotics...
2017: Frontiers in Cellular and Infection Microbiology
https://www.readbyqxmd.com/read/28415697/down-regulation-of-interleukin-7-receptor-il-7r-contributes-to-central-nervous-system-demyelination
#11
Xudan Lei, Shijiao Cai, Yang Chen, Jianlin Cui, Yajie Wang, Zongjin Li, Yuhao Li
Interleukin 7 receptor (IL-7R) has been associated with the pathogenesis of multiple sclerosis (MS), though the mechanisms are not clear. Because myelin expression is highly conserved between zebrafish and mammals, zebrafish have become an ideal model for studying demyelination. We used a transgenic (Tg; mbp:nfsB-egfp) zebrafish line in which oligodendrocytes expressed green fluorescent protein (GFP) from the larval stage to adulthood. Exposing adult transgenic zebrafish to metronidazole induced demyelination that resembled the morphological changes associated with the early stages of MS...
March 10, 2017: Oncotarget
https://www.readbyqxmd.com/read/28409342/zebrafish-as-a-model-of-kidney-disease
#12
Elvin E Morales, Rebecca A Wingert
Animal models have been an invaluable means to advance biomedical research as they provide experimental avenues for cellular and molecular investigations of disease pathology. The zebrafish (Danio rerio) is a good alternative to mammalian models that can be used to apply powerful genetic experimental methods normally used in invertebrates to answer questions about vertebrate development and disease. In the case of the kidney, the zebrafish has proven itself to be an applicable and versatile experimental system, mainly due to the simplicity of its pronephros, which contains two nephrons that possess conserved structural and physiological aspects with mammalian nephrons...
2017: Results and Problems in Cell Differentiation
https://www.readbyqxmd.com/read/28398482/cerkl-gene-knockout-disturbs-photoreceptor-outer-segment-phagocytosis-and-causes-rod-cone-dystrophy-in-zebrafish
#13
Shanshan Yu, Chang Li, Lincoln Biswas, Xuebin Hu, Fei Liu, James Reilly, Xiliang Liu, Ying Liu, Yuwen Huang, Zhaojing Lu, Shanshan Han, Lei Wang, Jing Yu Liu, Tao Jiang, Xinhua Shu, Fulton Wong, Zhaohui Tang, Mugen Liu
In humans, CERKL mutations cause widespread retinal degeneration: early dysfunction and loss of rod and cone photoreceptors in the outer retina and, progressively, death of cells in the inner retina. Despite intensive efforts, the function of CERKL remains obscure and studies in animal models have failed to clarify the disease mechanism of CERKL mutations. To address this gap in knowledge, we have generated a stable CERKL knockout zebrafish model by TALEN technology and a 7bp deletion in CERKL cDNA that caused the premature termination of CERKL...
April 7, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28393070/assessment-of-toxicological-perturbations-and-variants-of-pancreatic-islet-development-in-the-zebrafish-model
#14
Karilyn E Sant, Haydee M Jacobs, Jiali Xu, Katrina A Borofski, Larry G Moss, Jennifer B Moss, Alicia R Timme-Laragy
The pancreatic islets, largely comprised of insulin-producing beta cells, play a critical role in endocrine signaling and glucose homeostasis. Because they have low levels of antioxidant defenses and a high perfusion rate, the endocrine islets may be a highly susceptible target tissue of chemical exposures. However, this endpoint, as well as the integrity of the surrounding exocrine pancreas, is often overlooked in studies of developmental toxicology. Disruption of development by toxicants can alter cell fate and migration, resulting in structural alterations that are difficult to detect in mammalian embryo systems, but that are easily observed in the zebrafish embryo model (Danio rerio)...
September 2016: Toxics
https://www.readbyqxmd.com/read/28391149/multi-organ-toxicity-induced-by-fine-particulate-matter-pm2-5-in-zebrafish-danio-rerio-model
#15
Junchao Duan, Hejing Hu, Yannan Zhang, Lin Feng, Yanfeng Shi, Mark R Miller, Zhiwei Sun
The fine particulate matter (PM2.5) in air pollution is a major public health concern and now known to contribute to severe diseases, therefore, a comprehensive understanding of PM2.5-induced adverse effects in living organisms is needed urgently. This study was aimed to evaluate the toxicity of PM2.5 on multi-organ systems in a zebrafish (Danio rerio) model. The embryonic toxicity induced by PM2.5 was demonstrated by an increase in mortality and inhibition of hatching rate, in a dose- and time-dependent manner...
April 5, 2017: Chemosphere
https://www.readbyqxmd.com/read/28386438/expression-and-function-of-atp-dependent-potassium-channels-in-zebrafish-islet-%C3%AE-cells
#16
Christopher H Emfinger, Alecia Welscher, Zihan Yan, Yixi Wang, Hannah Conway, Jennifer B Moss, Larry G Moss, Maria S Remedi, Colin G Nichols
ATP-sensitive potassium channels (KATP channels) are critical nutrient sensors in many mammalian tissues. In the pancreas, KATP channels are essential for coupling glucose metabolism to insulin secretion. While orthologous genes for many components of metabolism-secretion coupling in mammals are present in lower vertebrates, their expression, functionality and ultimate impact on body glucose homeostasis are unclear. In this paper, we demonstrate that zebrafish islet β-cells express functional KATP channels of similar subunit composition, structure and metabolic sensitivity to their mammalian counterparts...
February 2017: Royal Society Open Science
https://www.readbyqxmd.com/read/28379616/from-fish-bowl-to-bedside-the-power-of-zebrafish-to-unravel-melanoma-pathogenesis-and-discover-new-therapeutics
#17
REVIEW
Ellen van Rooijen, Maurizio Fazio, Leonard I Zon
Melanoma is the most aggressive and deadliest form of skin cancer. A detailed knowledge of the cellular, molecular and genetic events underlying melanoma progression is highly relevant to diagnosis, prognosis and risk stratification, and the development of new therapies. In the last decade, zebrafish have emerged as a valuable model system for the study of melanoma. Pathway conservation, coupled with the availability of robust genetic, transgenic and chemical tools, has made the zebrafish a powerful model for identifying novel disease genes, visualizing cancer initiation, interrogating tumor-microenvironment interactions and discovering new therapeutics that regulate melanocyte and melanoma development...
April 5, 2017: Pigment Cell & Melanoma Research
https://www.readbyqxmd.com/read/28365779/personalized-genome-sequencing-coupled-with-ipsc-technology-identifies-gtdc1-as-a-gene-involved-in-neurodevelopmental-disorders
#18
Irene Aksoy, Kagistia H Utami, Cecilia L Winata, Axel M Hillmer, Sigrid L Rouam, Sylvain Briault, Sonia Davila, Lawrence W Stanton, Valere Cacheux
The cellular and molecular mechanisms underlying neurodevelopmental conditions such as autism spectrum disorders have been studied intensively for decades. The ability to generate patient-specific induced pluripotent stem cells (iPSCs) now offers a novel strategy for modelling human diseases. Recent studies have reported the derivation of iPSCs from patients with neurological disorders. The key challenge remains the demonstration of disease-related phenotypes and the ability to model the disease. Here we report a case study with signs of neurodevelopmental disorders (NDDs) harbouring chromosomal rearrangements that were sequenced using long-insert DNA paired-end tag (DNA-PET) sequencing approach...
January 15, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28359144/mind-bomb-binding-partner-ranbp9-plays-a-contributory-role-in-retinal-development
#19
Kyeong-Won Yoo, Maivannan Thiruvarangan, Yun-Mi Jeong, Mi-Sun Lee, Sateesh Maddirevula, Myungchull Rhee, Young-Ki Bae, Hyung-Goo Kim, Cheol-Hee Kim
Ran-binding protein family member, RanBP9 has been reported in various basic cellular mechanisms and neuropathological conditions including schizophrenia. Previous studies have reported that RanBP9 is highly expressed in the mammalian brain and retina; however, the role of RanBP9 in retinal development is largely unknown. Here, we present the novel and regulatory roles of RanBP9 in retinal development of a vertebrate animal model, zebrafish. Zebrafish embryos exhibited abundant expression of ranbp9 in developing brain tissues as well as in the developing retina...
March 28, 2017: Molecules and Cells
https://www.readbyqxmd.com/read/28351972/erbb2-regulates-autophagic-flux-to-modulate-the-proteostasis-of-app-ctfs-in-alzheimer-s-disease
#20
Bo-Jeng Wang, Guor Mour Her, Ming-Kuan Hu, Yun-Wen Chen, Ying-Tsen Tung, Pei-Yi Wu, Wen-Ming Hsu, Hsinyu Lee, Lee-Way Jin, Sheng-Ping L Hwang, Rita P-Y Chen, Chang-Jen Huang, Yung-Feng Liao
Proteolytic processing of amyloid precursor protein (APP) C-terminal fragments (CTFs) by γ-secretase underlies the pathogenesis of Alzheimer's disease (AD). An RNA interference screen using APP-CTF [99-residue CTF (C99)]- and Notch-specific γ-secretase interaction assays identified a unique ErbB2-centered signaling network that was predicted to preferentially govern the proteostasis of APP-C99. Consistently, significantly elevated levels of ErbB2 were confirmed in the hippocampus of human AD brains. We then found that ErbB2 effectively suppressed autophagic flux by physically dissociating Beclin-1 from the Vps34-Vps15 complex independent of its kinase activity...
April 11, 2017: Proceedings of the National Academy of Sciences of the United States of America
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