News Archive

Lab News

June 2018

Jacob Cain, a postdoctoral fellow in the Weimer laboratory, received the 2018 Dr. Ben Perryman Sanford Research Employee of the Year award. The recipient of this award demonstrates exceptional work performance, productivity, and lives the Sanford values every day.

June 2018

The immunosuppressant mycophenolate has been used for the treatment of autoimmune neurological diseases. As part of a clinical safety and tolerability study published in JIMD Reports, Jill Weimer’s research team analyzed biomarkers in CLN3-Batten disease patients before and after receiving short-term administration of mycophenolate. The treatment was well tolerated and future work will focus on the influence of long-term mycophenolate treatment on disease symptoms. "Short-term administration of mycophenolate is well-tolerated in CLN3 disease"

May 2018 Jill Weimer received an award from Recursion Pharmaceuticals for $396,000 to partner on a project titled, "Screening novel autophagy compounds for the treatment of multiple forms of Batten disease". This project will perform pre-clinical testing for pharmaceutical and small molecules targets for CLN2 and CLN8 Batten disease identified by Recursion via in vitro high throughput screening.

April 2018 Jill Weimer gave an invited seminar to the Oregon National Primate Research Faculty at Oregon Health & Science University. She presented updates on her research program's work on both Batten disease and Neurofibromatosis Type 1.

April 2018 Jon Brudvig, USD PhD student in the Weimer lab, successfully defended his thesis dissertation "Molecular roles of Myristoylated Alanine-Rich C Kinase Substrate (MARCKS) in neurite dynamics".

April 2018 Jill Weimer was invited to be part of the LabRoots, Inc. 6th Annual Neuroscience virtual conference.The goal of this online conference is to explore the mechanisms of neural function from the molecular to the network level in health and disease. World-renowned experts discussed the dynamics of brain function at various levels of consciousness, the mechanisms of functional networks and their modulation, and neuronal plasticity in recovery, as well as therapeutic approaches for symptomological amelioration. Jill presented on the Weimer team's Batten disease research in partnership with the CureBatten research team.

April 2018 Jill Weimer recently traveled to Iowa State University to meet with collaborator, Jinoh Kim. The Weimer lab works with Dr. Kim's team to study cellular trafficking of secretory and transmembrane proteins. While in Ames, Dr. Weimer also presented on her lab's work on rare diseases, highlighting successes in therapy development and generating of novel animal models of rare diseases.

April 2018 Jill Weimer, David Pearce, and Kevin Francis attended board meetings for the Charlotte and Gwenyth Gray Foundation and the Sebastian Velona Foundation in Santa Monica, CA.

March 2018 Jon Brudvig, USD BBS graduate student in the Weimer lab, received the inaugural CGSO (CBBRE Graduate Student Organization) Honorary Award for Excellence in Research, Service, and Leadership.This award honors undergraduate and graduate students for research contributions to their field, service contributions to the CGSO and CBBRe, and demonstration of overall leadership potential.

March 2018 Jill Weimer recently served on the NIH Neurodevelopment, Synaptic Plasticity and Neurodegeneration ZRG1 F03A-N study section. This review panel focuses on NIH F30 MD-PhD, F31 PhD and F32 postdoctoral trainee fellowship applications.

March 2018 Jill Weimer published a manuscript in Molecular Neurobiology describing a novel interaction between the protein MARCKS and the Netrin signaling complex. This work uncovers a novel role of MARCKS signaling in mediating axonal pathfinding in the developing cerebral cortex. “MARCKS is necessary for Netrin-DCC signaling and corpus callosum formation”.

March 2018 Jill Weimer received a third year of funding from the Charlotte and Gwenyth Gray Foundation to oversee collaborative efforts focused on development of novel therapies for the treatment of CLN6-Batten Disease.This multi-site program grant focuses on design and testing of therapies in the area of gene therapy, small molecules/repurposed drugs, RNA modulation, and use of stem cells to model human disease.

January 2018 Dr. Jill Weimer gave an invited seminar at LA Biomed - an affiliate of UCLA.  Her talk was titled "Innovative approaches for the study of rare pediatric diseases".

January 2018 Dr. Jill Weimer received a grant for $334,000 from the Sebastian Velona Foundation, which is funded by several foundations, title "scAAV9-CLN8 Gene Therapy Program".  This program focuses on the preclinical efficacy studies for a novel AAV9-CLN8 gene therapy program for the treatment of CLN8-Batten disease patients.
 
January 2018 Jill Weimer along with Ben Forred presented to students at Augustana University about rare disease research and the work being done at Sanford Research and with the Coordination of Rare Diseases at Sanford (CoRDS).
 
January 2018 Dr. Jill Weimer attended a board meeting for the Charlotte and Gwenyth Gray Foundation in Santa Monica, CA. 
 
December 2017 Dr. Jill Weimer and Sarah Hague organized a research conference in Bethesda, MD focused on management of CLN1 and CLN2 Batten Disease.  The conference brought together Batten disease researchers, families, and other academic researchers to discuss how to continue to move Batten research forward. “Batten Disease: 2017 Update of Translational Research for Management of INCL/LINCL”
 
December 2017 Dr. Jill Weimer was the keynote speaker at the annual symposium on Lysosomal Storage Disorders in Los Angeles, CA.  This conference, held as a collaborative event between UCLA, Cedars-Sinai and UC, Irvine, is focused on molecular diagnosis of lysosomal storage disorders and breakthroughs in therapies from the bench and bedside.  Dr. Weimer spoke about her lab's work developing therapies for Batten disease.
 
November 2017 Undergraduate students Kayla Knutson and McKayla Poppens, from the Weimer Lab, presented posters at the annual MidBrains Neuroscience Symposium in St. Paul. Kayla received the best poster award.
 
October 2017 Dr. Jill Weimer gave an invited presentation at the first annual Children's Tumor Foundation Synodos Network Meeting in Palm Beach, FL. A synodos is a multidisciplinary group of scientists and clinicians working together to solve a unique problem. Dr. Weimer co-leads a synodos for neurofibromatosis type 1 (NF1) focused on developing an NF1 animal model for preclinical drug testing. "Synodos for NF1: an innovative new porcine model of NF1"
 
September 2017 Dr. Jill Weimer gave an invited presentation on her laboratory's gene therapy program at the inaugural Charles River World Congress on Animal Models in Drug Discovery in Boston, MA. This conference discussed how clinical data is used to develop new animal models, tools and developing novel therapies: treatment of rare pediatric diseases"
 
September 2017 Dr. Jill Weimer and Ben Forred represented Sanford Research and the CoRDS registry at the annual Global Genes Summit in Irvine, CA. They interacted with patient advocacy groups, other researchers, and industry partners to promote Sanford's activities in rare disease research and to initiate new projects. Ben presented to a small group of potential partners on the benefits of working with CoRDS for their disease registry.
 
September 2017 Dr. Jill Weimer gave and invited presentation at the annual International Conference on Rare Diseases & Orphan Drugs in Beijing, China. This conference brings together individuals active in rare diseases and/or orphan drugs across areas of healthcare, research, patient advocacy and public policy. Dr. Pearce presented on her laboratory's work on Batten disease that has led to a CLN6 gene therapy program in partnership with Nationwide Children's Hospital.
 
September 2017 Dr. Jill Weimer co-authored a research article published in the Journal of Histochemistry and Cytochemistry validating immunohistochemical markers in pig tissue in anticipation of assessing pathologies in transgenic pigs modeling neurofibromatosis type 1 (NF1). “Immunohistochemical Markers for Prospective Studies in Neurofibromatosis-1 Porcine Models”
 
August 2017 Faculty, students and staff from the Weimer lab attended the annual Neurobehavioral Research Symposium for the Center for Brain and Behavior Research (CBBRe) at the University of South Dakota. Jon Brudvig, PhD Student in the Weimer lab, and Tom Hudson presented posters.
 
August 2017 Drs. Jill Weimer and Emily Griese authored a research article published in South Dakota Medicine investigating student attitudes regarding science and participation in informal science experiences. "Midwest science festival: exploring students' and parents' participation in and attitudes toward science".
 
August 2017 Dr. Jill Weimer received a non-research grant for USD from the NCHHD for her project "T35-Developmental Research Program from Medical Students".
 
July 2017 Dr. Jill Weimer was a co-author on a collaborative research article with co-workers Drs. David Pearce, Alexei Savinov, and Attila Kovacs published in Biochimica et Biphysica Acta investigating systemic effects such as infertility and immune dysfunction due to ataxia telangiectasia using a novel porcine model. "Genetic Ataxia Telangiectasia porcine model phenocopies the multisystemic features of the human disease" https://www.ncbi.nlm.nih.gov/pubmed/28746835
 
July 2017 Dr. Jill Weimer was interviewed by Dr. Drew on his Weekly Infusion podcast. Dr. Weimer discussed her ongoing gene therapy studies for Batten Disease, a pediatric neurodegenerative disease. http://drdrew.com/2017/wi-38-inside-battens-disease/
 
July 2017 Dr. Jill Weimer served on the Neurodevelopment, Synaptic Plasticity, and Neurodegeneration study section panel (F03A) for the National Institutes of Health (NIH). This study section reviews F30, F31, and F32 pre- and post-doctoral training fellowship applications.
 
June 2017 Dr. Jill Weimer received the 2017 Distinguished Researcher Award from the Sanford School of Medicine Department of Pediatrics. The Distinguished Researcher Award is given to a faculty member who has made significant contributions to the advancement of an area of biomedical, behavioral, or medical research, as evidenced by publications, national/international recognition, sustained record of competitive grant support by intramural and extramural agencies, and other scholarly activity while at the University of South Dakota Sanford School of Medicine.
 
May 2017 Dr. Jill Weimer attended the World Orphan Drug Congress in Washington DC. During this meeting, Dr. Weimer networked with a number of pharmaceutical and biotechnology companies to help foster relationships with Sanford Research laboratories.
 
May 2017 Dr. Tyler Johnson, a postdoctoral fellow in the Weimer lab, received a travel award from the NCL-Foundation to attend the Juvenile Neuronal Ceroid Lipofuscinosis Young Investigator Symposium in Hamburg, Germany.
 
May 2017 Drs. Jill Weimer and David Pearce published a research article in PLoS One characterizing a new mouse model of Cln2-infantile Batten disease which can be used for preclinical screening of nonsense suppression drugs. "A tailored mouse model of CLN2 disease: A nonsense mutant for testing personalized therapies".
 
April 2017 Jill Weimer, PhD, spoke at a virtual Children's Tumor Foundation (CTF) Town Hall. This event, attended by families of Neurofibromatosis and Schwannoma patients and members of the CTF's board and staff, provides a community forum to discuss updates related to the work funded through the CTF.
 
April 2017 A team of Sanford physicians and scientists led by Drs. Megan Landsverk, Kyle Roux, and Jill Weimer published a research article in American Journal of Medical Genetics that investigated the molecular consequences of PHOX2B mutations in patients with congenital central hypoventilation syndrome. "Nonsense pathogenic variants in exon 1 of PHOX2B lead to translational reinitiation in congenital central hypoventilation syndrome".
 
March 2017 Jill Weimer, PhD, along with Steven Powell, MD, presented to participants of the Leadership Sioux Falls course, organized by the Sioux Falls Area Chamber of Commerce. This program fosters the development of leadership in the greater Sioux Falls community. Dr. Weimer spoke about the basic and preclinical research going on in rare diseases at Sanford Research.
 
January 2017, Dr. Jill Weimer served on the Translational Research in Pediatrics and Obstetric Pharmacology and Therapeutics grant study section for the National Institutes of Health.
 
December 2016, Dr. Jill Weimer was an invited speaker at Dordt College in Sioux Center, IA. Dr. Weimer spoke to undergraduate science majors about the strategies used to study rare diseases and training opportunities available at Sanford Research for undergraduate and graduates students.
 
December 2016, Jon Brudvig, a PhD student in the Weimer lab, recently received a Trainee Research Grant from the University of South Dakota Center for Brain and Behavioral Research (CBBRe) for his project entitled "Novel Roles of MARCKS in Cortical Development and Midline Axon Crossing." CBBRe Research Training Grants provide trainees with funding for their research project as well as experience in writing and reviewing grants.
 
November 2016, Dr. Weimer was invited to present her work on mechanisms regulating the pathogenesis of CLN6-Batten disease and development of effective therapies to the Department of Pathology at Boston Children's Hospital of Harvard Medical School in Boston, MA.
 
November 2016, Dr. Weimer organized and presented during a mini-symposium entitled "Building the cerebral cortex: Mechanisms that mediate migration, specification, and axonal outgrowth" as part of the 2016 Society for Neuroscience Meeting in San Diego, CA.
 
October 2016, Dr. Weimer gave a presentation entitled "Team science to advance therapeutic development for rare diseases" as part of the Advancing Rare Disease Drug Discovery Symposium sponsored by Charles River Laboratories in New York, NY.
 
October 2016, Dr. Jacob Cain, a postdoctoral fellow in the Weimer laboratory, received a travel award to attend the 2016 Neuronal Ceroid Lipofuscinosis International Congress in Boston, MA.
 
August 2016, Dr. Weimer presented research updates from her laboratory on CLN5, CLN6, CLN7, and CLN8-Batten disease to families at the 2016 Batten Disease Support and Research Association Family Conference in St. Louis, MO.
 
August 2016, Dr. Jacob Cain, a postdoctoral fellow in Dr. Jill Weimer’s lab, received a 1 year grant from the Batten Disease Support and Research Association. This research will focus on development of a novel mouse model that allows for cell specific introduction of a disease causing mutation associated with CLN3-Batten disease.
 
July 2016, Dr. Jill Weimer and a member of her team, Katherine White, recently published a study revealing that increased anxiety rate in obese patients may be caused by an interaction between obesity and a very common human infection, which results in immunological changes in the brain. This study, entitled “Diet-induced obesity prolongs neuroinflammation and recruits CCR2+ monocytes to the brain following herpes simplex virus (HSV)-1 latency in mice,” was published in the journal Brain, Behavior, and Immunity in collaboration with colleagues Drs. Patti Sheridan and Scott Hutton at the University of North Carolina.
 
August 2016, Dr. Jill Weimer recently received an administrative supplement for her R01 entitled “Novel mechanisms for distal transport in developing and mature neurons” funded through the National Institute Of Neurological Disorders And Stroke of the National Institutes of Health. This $100,000 award (direct and indirect) will allow the Weimer team to investigate whether sex influence the age of onset, rate of decline and response to drug treatment in CLN6-Batten disease patients. Preliminary studies from the Weimer lab suggest that, in a mouse model of CLN6-Batten disease, male present earlier with disease symptoms than female littermates – which could significantly shift the window for administration of any disease therapy.
 
June 2016, Dr. Jill Weimer recently organized and hosted the 5th Annual It’s All About Science Festival at the Sanford Center which was attended by nearly 4000 community members. The event is organized annually by Sanford Research in partnership with area biotech companies, academic affiliates and non-profit organization. Through her STEM education efforts, Dr. Weimer also currently serves as the Chairwomen of the Kirby Science Discovery Center Board and is a member of the Board of Trustees at the Washington Pavilion.
 
May 2016, USD graduate student Jon Brudvig has won a 2016 National Defense Science and Engineering Graduate (NDSEG) Fellowship. Jon is a second year PhD student in the Basic Biomedical Sciences program. He is completing his dissertation research in the Weimer Lab at Sanford Research, studying the role of cell polarity proteins in development of the cerebral cortex. The NDSEG is a highly competitive predoctoral fellowship funded by the Department of Defense, and supports individuals who have demonstrated the ability and special aptitude for advanced training in science and engineering.
 
May 2016, Dr. Jill Weimer was recently elected to the Alumnae Council for the School of Medicine and Dentistry of the University of Rochester in Rochester NY. Dr. Weimer received her BA, MS, and PhD from the University of Rochester and was the very first graduate student member appointed to the council while she was at student at the UR.
 
April 2016, Jon Brudvig, a graduate student in the Weimer lab, has received a travel award from the Center for Brain and Behavior Research (CBBRe) at USD to attend the 2016 Neural Development Gordon Research Conference in Newport, RI. Jon has also been selected to attend a pre- "Neural Development" Gordon Research Seminar (GRS) for graduate and postdoctoral trainees. Jon is a second year PhD candidate in the Basic Biomedical Science Program at USD.
 
March 2016, Rachel Laufmann, a senior at USD and full-time technician in the Weimer lab, has been awarded the 2016 Undergraduate Research Excellence Award from the University of South Dakota. This prestigious award recognizes 3+ years’ of research that Rachel conducted both as an REU/SPUR scholar and as a part of her undergraduate honors thesis. The award includes a grant of $500, a featured month in the USD 2017 Office of Research and Sponsored Programs calendar, and a framed citation for her accomplishments.
 
March 2016, Dr. Jill Weimer gave an invited lecture at the 2016 American Society for Neurochemistry in Denver March 19-23rd. Dr. Weimer’s presentation will be a part of a special panel entitled “Old Friends and New Roles: Brain Patterning and Signal Transduction.” The goal of the American Society for Neurochemistry is to advance and promote the science of neurochemistry and related neurosciences and to increase and enhance neurochemical knowledge.
 
November 2015, Dr. Jill Weimer gave two invited seminars on her CLN6-Batten disease research. The first was in the Department of Pharmacology at the University of Arizona in Tucson. The second was at the Barrow Neurological Institute at Arizona State University in Phoenix.
 
November 2015, Dr. Jill Weimer and a USD BBS graduate student with her team, Jon Brudvig, recently published a review article entitled “X MARCKS the spot: myristoylated alanine-rich C kinase substrate in neuronal function and disease” in the journal Frontiers in Cellular Neuroscience.
 
November 2015, Dr. Jill Weimer presented updates on her Batten disease research at Young Harris College in Georgia.
 
October 2015, Dr. Jill Weimer was recently interviewed for an episode of The Doctors TV show regarding her work on Batten disease in partnership with the Charlotte and Gwenyth Gray Foundation. This taped episode aired October 12th.
 
October 2015, Jon Brudvig, a BBS PhD student in the Weimer lab, has received a travel award from the Center for Brain Behavior Research to attend the Society for Neuroscience meeting in Chicago to present his research and to attend a stem cell training course.
 
October 2015, Dr. Jill Weimer received a research grant for nearly $440,000 from The Charlotte and Gwenyth Gray Foundation for her research on finding a cure or treatment for a rare variant form of Batten disease. http://www.sanfordhealth.org/newsroom/2015/10/Sanford-scientist-lands-grant-to-support-Batten-disease-research
 
October 17-21st: The Weimer team attended and presented at the international Society for Neuroscience
meeting in Chicago, IL. 
 
In September 2015, Jacob Cain, a postdoctoral fellow in the Weimer lab, was awarded a pilot grant from the Center for Cancer Biology (NIH funded COBRE) program at Sanford Research
 
September 11, 2015: Dr. Jill Weimer was a special invited speaker at Nationwide Children's Hospital in Columbus OH where she presented on “Faulty regulation of the CCK complex in Batten’s disease and its role in vesicular transport”
 
August 2014: Weimer lab member Ryan O’Toole departed the lab to start on his next adventure as a graduate student at Dartmouth University
 
May 5th: Jill presented at the Second Annual Center for Pediatric Research (NIH CoBRE Program) Symposium held at Sanford Research
 
June 6-9th: Jill attended the Children’s Tumor Foundation’s annual research conference in Monterey CA
 
June 1st: The Weimer team has been joined by 5 new summer interns (Keegan Mechels, Ryan Patrick, Caeden Tinklenberg, Brittany Kuhl, and Darren DeNeui)
 
May 18-19th: Jill presented at the first annual Sanford Consortium/Sanford Research Stem Cell Symposium at University of California – San Diego