Hugo J Bellen

Summary

Affiliation: Baylor College of Medicine
Country: USA

Publications

  1. Haelterman N, Jiang L, Li Y, Bayat V, Sandoval H, Ugur B, et al. Large-scale identification of chemically induced mutations in Drosophila melanogaster. Genome Res. 2014;24:1707-18 pubmed publisher
    ..Hence, our findings demonstrate that combining rough mapping with WGS dramatically expands the toolkit necessary for assigning function to genes. ..
  2. Senturk M, Lin G, Zuo Z, Mao D, Watson E, Mikos A, et al. Ubiquilins regulate autophagic flux through mTOR signalling and lysosomal acidification. Nat Cell Biol. 2019;21:384-396 pubmed publisher
    ..Hence, our studies reveal a conserved role for ubiquilins as regulators of autophagy by controlling vacuolar H+-ATPase activity and mTOR signalling. ..
  3. Shah P, Link N, Jang G, Sharp P, Zhu T, Swaney D, et al. Comparative Flavivirus-Host Protein Interaction Mapping Reveals Mechanisms of Dengue and Zika Virus Pathogenesis. Cell. 2018;175:1931-1945.e18 pubmed publisher
    ..Thus, comparative flavivirus-host PPI mapping provides biological insights and, when coupled with in vivo models, can be used to unravel pathogenic mechanisms. ..
  4. Lin G, Wang L, Marcogliese P, Bellen H. Sphingolipids in the Pathogenesis of Parkinson's Disease and Parkinsonism. Trends Endocrinol Metab. 2019;30:106-117 pubmed publisher
    ..We propose that disruption of ceramide metabolism may affect endolysosomal and mitochondrial function, and plays an important role in PD/parkinsonism. ..
  5. Ansar M, Chung H, Taylor R, Nazir A, Imtiaz S, Sarwar M, et al. Bi-allelic Loss-of-Function Variants in DNMBP Cause Infantile Cataracts. Am J Hum Genet. 2018;103:568-578 pubmed publisher
    ..E-cadherin has an important role in lens vesicle separation and lens epithelial cell survival in humans. We therefore conclude that DNMBP loss-of-function variants cause infantile-onset cataracts in humans. ..
  6. Yamamoto S, Jaiswal M, Charng W, Gambin T, Karaca E, Mirzaa G, et al. A drosophila genetic resource of mutants to study mechanisms underlying human genetic diseases. Cell. 2014;159:200-214 pubmed publisher
    ..This bidirectional synergism between fly genetics and human genomics facilitates the functional annotation of evolutionarily conserved genes involved in human health. ..
  7. Marcogliese P, Shashi V, Spillmann R, STONG N, Rosenfeld J, Koenig M, et al. IRF2BPL Is Associated with Neurological Phenotypes. Am J Hum Genet. 2018;103:245-260 pubmed publisher
    ..Taken together, our results show that IRF2BPL and pits are required in the nervous system in humans and flies, and their loss leads to a range of neurological phenotypes in both species. ..
  8. Lin G, Lee P, Chen K, Mao D, Tan K, Zuo Z, et al. Phospholipase PLA2G6, a Parkinsonism-Associated Gene, Affects Vps26 and Vps35, Retromer Function, and Ceramide Levels, Similar to ?-Synuclein Gain. Cell Metab. 2018;: pubmed publisher
    ..Similar defects are observed upon loss of vps26 or vps35 or overexpression of ?-synuclein, indicating that these defects may be common in Parkinson disease. ..
  9. Ugur B, Chen K, Bellen H. Drosophila tools and assays for the study of human diseases. Dis Model Mech. 2016;9:235-44 pubmed publisher
    ..These assays provide us with simple yet powerful tools to study the pathogenic mechanisms associated with human disease-causing genes. ..

More Information

Publications25

  1. Liu L, Zhang K, Sandoval H, Yamamoto S, Jaiswal M, Sanz E, et al. Glial lipid droplets and ROS induced by mitochondrial defects promote neurodegeneration. Cell. 2015;160:177-90 pubmed publisher
  2. Ugur B, Bao H, Stawarski M, Duraine L, Zuo Z, Lin Y, et al. The Krebs Cycle Enzyme Isocitrate Dehydrogenase 3A Couples Mitochondrial Metabolism to Synaptic Transmission. Cell Rep. 2017;21:3794-3806 pubmed publisher
    ..The data reveal conserved metabolic regulation of synaptic transmission via αKG. Our studies provide a synaptic role for αKG, a metabolite that has been proposed as a treatment for aging and neurodegenerative disorders. ..
  3. Wangler M, Yamamoto S, Chao H, Posey J, Westerfield M, Postlethwait J, et al. Model Organisms Facilitate Rare Disease Diagnosis and Therapeutic Research. Genetics. 2017;207:9-27 pubmed publisher
    ..We discuss how merging human genetics with model organism research guides experimental studies to solve these medical mysteries, gain new insights into disease pathogenesis, and uncover new therapeutic strategies. ..
  4. Chao H, Liu L, Bellen H. Building dialogues between clinical and biomedical research through cross-species collaborations. Semin Cell Dev Biol. 2017;70:49-57 pubmed publisher
  5. Lin G, Mao D, Bellen H. Amyotrophic Lateral Sclerosis Pathogenesis Converges on Defects in Protein Homeostasis Associated with TDP-43 Mislocalization and Proteasome-Mediated Degradation Overload. Curr Top Dev Biol. 2017;121:111-171 pubmed publisher
  6. Bellen H, Yamamoto S. Morgan's legacy: fruit flies and the functional annotation of conserved genes. Cell. 2015;163:12-4 pubmed publisher
    ..Flies continue to offer great opportunities, including studies in the field of functional genomics. ..
  7. Wang S, Bellen H. The retromer complex in development and disease. Development. 2015;142:2392-6 pubmed publisher
    ..Here, and in the accompanying poster, we provide an overview of the molecular and cellular mechanisms of retromer-mediated protein trafficking, highlighting key examples of retromer function in vivo. ..
  8. Wangler M, Yamamoto S, Bellen H. Fruit flies in biomedical research. Genetics. 2015;199:639-53 pubmed publisher
    ..We would like to promote a better collaboration between Drosophila geneticists/biologists and human geneticists/bioinformaticians/clinicians, as it would benefit both fields and significantly impact the research on human diseases. ..
  9. Wang J, Al Ouran R, Hu Y, Kim S, Wan Y, Wangler M, et al. MARRVEL: Integration of Human and Model Organism Genetic Resources to Facilitate Functional Annotation of the Human Genome. Am J Hum Genet. 2017;100:843-853 pubmed publisher
  10. Liu L, MacKenzie K, Putluri N, Maletic Savatic M, Bellen H. The Glia-Neuron Lactate Shuttle and Elevated ROS Promote Lipid Synthesis in Neurons and Lipid Droplet Accumulation in Glia via APOE/D. Cell Metab. 2017;26:719-737.e6 pubmed publisher
  11. Senturk M, Bellen H. Genetic strategies to tackle neurological diseases in fruit flies. Curr Opin Neurobiol. 2018;50:24-32 pubmed publisher
    ..We highlight how systematic and combinatorial approaches based on recently established methods provide us with integrated tool sets that can be applied to the study of neurodevelopmental and neurodegenerative disorders. ..
  12. Landrock K, Sullivan P, Martini Stoica H, Goldstein D, Graham B, Yamamoto S, et al. Pleiotropic neuropathological and biochemical alterations associated with Myo5a mutation in a rat Model. Brain Res. 2018;1679:155-170 pubmed publisher
  13. Tan K, Haelterman N, Kwartler C, Regalado E, Lee P, Nagarkar Jaiswal S, et al. Ari-1 Regulates Myonuclear Organization Together with Parkin and Is Associated with Aortic Aneurysms. Dev Cell. 2018;45:226-244.e8 pubmed publisher
    ..In addition, SMCs obtained from patients display aberrant nuclear morphology. Hence, ARIH1 is critical in anchoring myonuclei to the cytoskeleton. ..
  14. Yoon W, Sandoval H, Nagarkar Jaiswal S, Jaiswal M, Yamamoto S, Haelterman N, et al. Loss of Nardilysin, a Mitochondrial Co-chaperone for ?-Ketoglutarate Dehydrogenase, Promotes mTORC1 Activation and Neurodegeneration. Neuron. 2017;93:115-131 pubmed publisher
    ..In summary, this study reveals a novel role for NRD1 as a mitochondrial co-chaperone for OGDH and provides a mechanistic link between mitochondrial metabolic dysfunction, mTORC1 signaling, and impaired autophagy in neurodegeneration. ..
  15. David Morrison G, Xu Z, Rui Y, Charng W, Jaiswal M, Yamamoto S, et al. WAC Regulates mTOR Activity by Acting as an Adaptor for the TTT and Pontin/Reptin Complexes. Dev Cell. 2016;36:139-51 pubmed publisher
    ..WAC promotes the interaction between TTT and Pontin/Reptin in an energy-dependent manner, thereby promoting mTORC1 activity by facilitating mTORC1 dimerization and mTORC1-Rag interaction. ..
  16. Charng W, Yamamoto S, Bellen H. Shared mechanisms between Drosophila peripheral nervous system development and human neurodegenerative diseases. Curr Opin Neurobiol. 2014;27:158-64 pubmed publisher
    ..In this review, we summarize and discuss how studies of Drosophila PNS and NMJ development have provided guidance in experimental approaches for these diseases. ..