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Tyler B Johnson

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Recent Articles
1.
Swier V, White K, Negrao de Assis P, Johnson T, Leppert H, Rechtzigel M, et al.
Clin Transl Sci . 2024 Jun; 17(6):e13858. PMID: 38932491
Cognitive or motor impairment is common among individuals with neurofibromatosis type 1 (NF1), an autosomal dominant tumor-predisposition disorder. As many as 70% of children with NF1 report difficulties with spatial/working...
2.
Leppert H, Anderson J, Timm K, Davoli C, Pratt M, Booth C, et al.
bioRxiv . 2023 Oct; PMID: 37790379
Lysosomal storage disorders (LSDs) are a genetically and clinically diverse group of diseases characterized by lysosomal dysfunction. Batten disease is a family of severe LSDs primarily impacting the central nervous...
3.
Swier V, White K, Johnson T, Wang X, Han J, Pearce D, et al.
Dis Model Mech . 2023 Jun; 16(8). PMID: 37305926
Mouse models of CLN3 Batten disease, a rare lysosomal storage disorder with no cure, have improved our understanding of CLN3 biology and therapeutics through their ease of use and a...
4.
Johnson T, Brudvig J, Likhite S, Pratt M, White K, Cain J, et al.
Front Genet . 2023 Apr; 14:1118649. PMID: 37035740
CLN3 disease, caused by biallelic mutations in the gene, is a rare pediatric neurodegenerative disease that has no cure or disease modifying treatment. The development of effective treatments has been...
5.
Holmes A, White K, Pratt M, Johnson T, Likhite S, Meyer K, et al.
Orphanet J Rare Dis . 2022 Nov; 17(1):411. PMID: 36369162
Background: CLN8-Batten disease (CLN8 disease) is a rare neurodegenerative disorder characterized phenotypically by progressive deterioration of motor and cognitive abilities, visual symptoms, epileptic seizures, and premature death. Mutations in CLN8...
6.
Brudvig J, Swier V, Johnson T, Cain J, Pratt M, Rechtzigel M, et al.
Biomark Insights . 2022 Oct; 17:11772719221107765. PMID: 36212622
Introduction: CLN3 Batten disease is a rare pediatric neurodegenerative lysosomal disorder caused by biallelic disease-associated variants in Despite decades of intense research, specific biofluid biomarkers of disease status have not...
7.
Swier V, White K, Johnson T, Sieren J, Johnson H, Knoernschild K, et al.
Neurotherapeutics . 2022 Sep; 19(6):1905-1919. PMID: 36100791
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 typically manifests around 2 to...
8.
Rechtzigel M, Meyerink B, Leppert H, Johnson T, Cain J, Ferrandino G, et al.
Front Neurosci . 2022 Jun; 16:834780. PMID: 35692423
Batten disease is unique among lysosomal storage disorders for the early and profound manifestation in the central nervous system, but little is known regarding potential neuron-specific roles for the disease-associated...
9.
White K, Nelvagal H, Poole T, Lu B, Johnson T, Davis S, et al.
Mol Ther Methods Clin Dev . 2021 Mar; 20:497-507. PMID: 33665223
Batten disease is a family of rare, fatal, neuropediatric diseases presenting with memory/learning decline, blindness, and loss of motor function. Recently, we reported the use of an AAV9-mediated gene therapy...
10.
Johnson T, Adamec J, Blum P
Bio Protoc . 2021 Mar; 10(7):e3570. PMID: 33659540
High magnetic field Fourier transform ion cyclotron resonance (FT-ICR) mass spectrometers provide extremely high mass resolution (resolving power of ~200,000 at 400 m/z) protein detection across a broad mass range,...