Search Results - "Johnson, Tyler B"

AAV9 Gene Therapy Increases Lifespan and Treats Pathological and Behavioral Abnormalities in a Mouse Model of CLN8-Batten Disease by Johnson, Tyler B., White, Katherine A., Brudvig, Jon J., Cain, Jacob T., Langin, Logan, Pratt, Melissa A., Booth, Clarissa D., Timm, Derek J., Davis, Samantha S., Meyerink, Brandon, Likhite, Shibi, Meyer, Kathrin, Weimer, Jill M.

“…CLN8 disease is a rare form of neuronal ceroid lipofuscinosis caused by biallelic mutations in the CLN8 gene, which encodes a transmembrane endoplasmic…”
Get full text

Early postnatal administration of an AAV9 gene therapy is safe and efficacious in CLN3 disease by Johnson, Tyler B, Brudvig, Jon J, Likhite, Shibi, Pratt, Melissa A, White, Katherine A, Cain, Jacob T, Booth, Clarissa D, Timm, Derek J, Davis, Samantha S, Meyerink, Brandon, Pineda, Ricardo, Dennys-Rivers, Cassandra, Kaspar, Brian K, Meyer, Kathrin, Weimer, Jill M

“…CLN3 disease, caused by biallelic mutations in the gene, is a rare pediatric neurodegenerative disease that has no cure or disease modifying treatment. The…”
Get full text

A novel porcine model of CLN3 Batten disease recapitulates clinical phenotypes by Swier, Vicki J, White, Katherine A, Johnson, Tyler B, Wang, Xiaojun, Han, Jimin, Pearce, David A, Singh, Ruchira, Drack, Arlene V, Pfeifer, Wanda, Rogers, Christopher S, Brudvig, Jon J, Weimer, Jill M

“…Mouse models of CLN3 Batten disease, a rare lysosomal storage disorder with no cure, have improved our understanding of CLN3 biology and therapeutics through…”
Get full text

Tracking sex-dependent differences in a mouse model of CLN6-Batten disease by Poppens, McKayla J, Cain, Jacob T, Johnson, Tyler B, White, Katherine A, Davis, Samantha S, Laufmann, Rachel, Kloth, Alexander D, Weimer, Jill M

“…CLN6-Batten disease is a rare neurodevelopmental disorder characterized pathologically by the accumulation of lysosomal storage material, glial activation and…”
Get full text

NF1+/ex42del miniswine model the cellular disruptions and behavioral presentations of NF1‐associated cognitive and motor impairment by Swier, Vicki J., White, Katherine A., Negrão de Assis, Pedro L., Johnson, Tyler B., Leppert, Hannah G., Rechtzigel, Mitchell J., Meyerholz, David K., Dodd, Rebecca D., Quelle, Dawn E., Khanna, Rajesh, Rogers, Christopher S., Weimer, Jill M.

“…Cognitive or motor impairment is common among individuals with neurofibromatosis type 1 (NF1), an autosomal dominant tumor‐predisposition disorder. As many as…”
Get full text

Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment by Rechtzigel, Mitchell J, Meyerink, Brandon L, Leppert, Hannah, Johnson, Tyler B, Cain, Jacob T, Ferrandino, Gavin, May, Danielle G, Roux, Kyle J, Brudvig, Jon J, Weimer, Jill M

“…Batten disease is unique among lysosomal storage disorders for the early and profound manifestation in the central nervous system, but little is known…”
Get full text

Intracranial delivery of AAV9 gene therapy partially prevents retinal degeneration and visual deficits in CLN6-Batten disease mice by White, Katherine A., Nelvagal, Hemanth R., Poole, Timothy A., Lu, Bin, Johnson, Tyler B., Davis, Samantha, Pratt, Melissa A., Brudvig, Jon, Assis, Ana B., Likhite, Shibi, Meyer, Kathrin, Kaspar, Brian K., Cooper, Jonathan D., Wang, Shaomei, Weimer, Jill M.

“…Batten disease is a family of rare, fatal, neuropediatric diseases presenting with memory/learning decline, blindness, and loss of motor function. Recently, we…”
Get full text

A human model of Batten disease shows role of CLN3 in phagocytosis at the photoreceptor–RPE interface by Tang, Cynthia, Han, Jimin, Dalvi, Sonal, Manian, Kannan, Winschel, Lauren, Volland, Stefanie, Soto, Celia A., Galloway, Chad A., Spencer, Whitney, Roll, Michael, Milliner, Caroline, Bonilha, Vera L., Johnson, Tyler B., Latchney, Lisa, Weimer, Jill M., Augustine, Erika F., Mink, Jonathan W., Gullapalli, Vamsi K., Chung, Mina, Williams, David S., Singh, Ruchira

“…Mutations in CLN3 lead to photoreceptor cell loss in CLN3 disease, a lysosomal storage disorder characterized by childhood-onset vision loss, neurological…”
Get full text

Therapeutic landscape for Batten disease: current treatments and future prospects by Johnson, Tyler B., Cain, Jacob T., White, Katherine A., Ramirez-Montealegre, Denia, Pearce, David A., Weimer, Jill M.

“…Batten disease (also known as neuronal ceroid lipofuscinoses) constitutes a family of devastating lysosomal storage disorders that collectively represent the…”
Get full text

Therapeutic efficacy of antisense oligonucleotides in mouse models of CLN3 Batten disease by Centa, Jessica L., Jodelka, Francine M., Hinrich, Anthony J., Johnson, Tyler B., Ochaba, Joseph, Jackson, Michaela, Duelli, Dominik M., Weimer, Jill M., Rigo, Frank, Hastings, Michelle L.

“…CLN3 Batten disease is an autosomal recessive, neurodegenerative, lysosomal storage disease caused by mutations in CLN3 , which encodes a lysosomal membrane…”
Get full text

Gene Therapy Corrects Brain and Behavioral Pathologies in CLN6-Batten Disease by Cain, Jacob T., Likhite, Shibi, White, Katherine A., Timm, Derek J., Davis, Samantha S., Johnson, Tyler B., Dennys-Rivers, Cassandra N., Rinaldi, Federica, Motti, Dario, Corcoran, Sarah, Morales, Pablo, Pierson, Christopher, Hughes, Stephanie M., Lee, Stella Y., Kaspar, Brian K., Meyer, Kathrin, Weimer, Jill M.

“…CLN6-Batten disease, a form of neuronal ceroid lipofuscinosis is a rare lysosomal storage disorder presenting with gradual declines in motor, visual, and…”
Get full text

Changes in motor behavior, neuropathology, and gut microbiota of a Batten disease mouse model following administration of acidified drinking water by Johnson, Tyler B., Langin, Logan M., Zhao, Jing, Weimer, Jill M., Pearce, David A., Kovács, Attila D.

“…CLN3 mutations cause the fatal neurodegenerative disorder, CLN3 Batten disease. The Cln3 −/− mouse model displays characteristic features of the human disease…”
Get full text

A tailored Cln3Q352X mouse model for testing therapeutic interventions in CLN3 Batten disease by Langin, Logan, Johnson, Tyler B., Kovács, Attila D., Pearce, David A., Weimer, Jill M.

“…CLN3 Batten disease (CLN3 disease) is a pediatric lysosomal storage disorder that presents with progressive blindness, motor and cognitive decline, seizures,…”
Get full text

Sex-split analysis of pathology and motor-behavioral outcomes in a mouse model of CLN8-Batten disease reveals an increased disease burden and trajectory in female Cln8mnd mice by Holmes, Andrew D, White, Katherine A, Pratt, Melissa A, Johnson, Tyler B, Likhite, Shibi, Meyer, Kathrin, Weimer, Jill M

“…Abstract Background CLN8-Batten disease (CLN8 disease) is a rare neurodegenerative disorder characterized phenotypically by progressive deterioration of motor…”
Get full text

A Novel Porcine Model of CLN2 Batten Disease that Recapitulates Patient Phenotypes by Swier, Vicki J., White, Katherine A., Johnson, Tyler B., Sieren, Jessica C., Johnson, Hans J., Knoernschild, Kevin, Wang, Xiaojun, Rohret, Frank A., Rogers, Christopher S., Pearce, David A., Brudvig, Jon J., Weimer, Jill M.

“…CLN2 Batten disease is a lysosomal disorder in which pathogenic variants in CLN2 lead to reduced activity in the enzyme tripeptidyl peptidase 1. The disease…”
Get full text

A multimodal approach to identify clinically relevant biomarkers to comprehensively monitor disease progression in a mouse model of pediatric neurodegenerative disease by Johnson, Tyler B., Brudvig, Jon J., Lehtimäki, Kimmo K., Cain, Jacob T., White, Katherine A., Bragge, Timo, Rytkönen, Jussi, Huhtala, Tuulia, Timm, Derek, Vihma, Maria, Puoliväli, Jukka T., Poutiainen, Pekka, Nurmi, Antti, Weimer, Jill M.

“…•Longitudinal neuroimaging reveals changes in Cln6nclf mice that correlate with changes reported in patients with Batten disease.•Brain region-specific…”
Get full text

Secretion and fusion of biogeochemically active archaeal membrane vesicles by Johnson, Tyler B., Mach, Collin, Grove, Ryan, Kelly, Robert, Van Cott, Kevin, Blum, Paul

“…Microbes belonging to the genus Metallosphaera oxidize sulfidic minerals. These organisms thrive at temperature extremes and are members of the archaeal phylum…”
Get full text

Author Correction: Characterization of a recurrent missense mutation in the forkhead DNA-binding domain of FOXP1 by Johnson, Tyler B., Mechels, Keegan, Anderson, Ruthellen H., Cain, Jacob T., Sturdevant, David A., Braddock, Stephen, Pinz, Hailey, Wilson, Mark A., Landsverk, Megan, Roux, Kyle J., Weimer, Jill M.

“…An amendment to this paper has been published and can be accessed via a link at the top of the paper…”
Get full text

Characterization of a recurrent missense mutation in the forkhead DNA-binding domain of FOXP1 by Johnson, Tyler B., Mechels, Keegan, Anderson, Ruthellen H., Cain, Jacob T., Sturdevant, David A., Braddock, Stephen, Pinz, Hailey, Wilson, Mark A., Landsverk, Megan, Roux, Kyle J., Weimer, Jill M.

“…Haploinsufficiency of Forkhead box protein P1 (FOXP1), a highly conserved transcription factor, leads to developmental delay, intellectual disability, autism…”
Get full text

Preparation, FPLC Purification and LC-FT-ICR-MS of Proteins by Johnson, Tyler B, Adamec, Jiri, Blum, Paul

“…High magnetic field Fourier transform ion cyclotron resonance (FT-ICR) mass spectrometers provide extremely high mass resolution (resolving power of ~200,000…”
Get full text