Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment
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 proteins. We demonstrate substantial overlap in the protein interactomes of thre...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2022-05-01
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| Series: | Frontiers in Neuroscience |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/articles/10.3389/fnins.2022.834780/full |
| _version_ | 1828224757914402816 |
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| author | Mitchell J. Rechtzigel Brandon L. Meyerink Brandon L. Meyerink Hannah Leppert Tyler B. Johnson Jacob T. Cain Gavin Ferrandino Danielle G. May Kyle J. Roux Kyle J. Roux Jon J. Brudvig Jon J. Brudvig Jill M. Weimer Jill M. Weimer |
| author_facet | Mitchell J. Rechtzigel Brandon L. Meyerink Brandon L. Meyerink Hannah Leppert Tyler B. Johnson Jacob T. Cain Gavin Ferrandino Danielle G. May Kyle J. Roux Kyle J. Roux Jon J. Brudvig Jon J. Brudvig Jill M. Weimer Jill M. Weimer |
| author_sort | Mitchell J. Rechtzigel |
| collection | DOAJ |
| description | 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 proteins. We demonstrate substantial overlap in the protein interactomes of three transmembrane Batten proteins (CLN3, CLN6, and CLN8), and that their absence leads to synaptic depletion of key partners (i.e., SNAREs and tethers) and altered synaptic SNARE complexing in vivo, demonstrating a novel shared etiology. |
| first_indexed | 2024-04-12T17:22:08Z |
| format | Article |
| id | doaj.art-fa0434a2a5f14c128f2774c04d0eb17d |
| institution | Directory Open Access Journal |
| issn | 1662-453X |
| language | English |
| last_indexed | 2024-04-12T17:22:08Z |
| publishDate | 2022-05-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neuroscience |
| spelling | doaj.art-fa0434a2a5f14c128f2774c04d0eb17d2022-12-22T03:23:26ZengFrontiers Media S.A.Frontiers in Neuroscience1662-453X2022-05-011610.3389/fnins.2022.834780834780Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic EnrichmentMitchell J. Rechtzigel0Brandon L. Meyerink1Brandon L. Meyerink2Hannah Leppert3Tyler B. Johnson4Jacob T. Cain5Gavin Ferrandino6Danielle G. May7Kyle J. Roux8Kyle J. Roux9Jon J. Brudvig10Jon J. Brudvig11Jill M. Weimer12Jill M. Weimer13Pediatrics and Rare Diseases Group, Sanford Research, Sioux Falls, SD, United StatesPediatrics and Rare Diseases Group, Sanford Research, Sioux Falls, SD, United StatesBasic Biomedical Sciences, Sanford School of Medicine at the University of South Dakota, Vermillion, SD, United StatesPediatrics and Rare Diseases Group, Sanford Research, Sioux Falls, SD, United StatesPediatrics and Rare Diseases Group, Sanford Research, Sioux Falls, SD, United StatesPediatrics and Rare Diseases Group, Sanford Research, Sioux Falls, SD, United StatesPediatrics and Rare Diseases Group, Sanford Research, Sioux Falls, SD, United StatesPediatrics and Rare Diseases Group, Sanford Research, Sioux Falls, SD, United StatesPediatrics and Rare Diseases Group, Sanford Research, Sioux Falls, SD, United StatesDepartment of Pediatrics, Sanford School of Medicine at the University of South Dakota, Vermillion, SD, United StatesPediatrics and Rare Diseases Group, Sanford Research, Sioux Falls, SD, United StatesDepartment of Pediatrics, Sanford School of Medicine at the University of South Dakota, Vermillion, SD, United StatesPediatrics and Rare Diseases Group, Sanford Research, Sioux Falls, SD, United StatesDepartment of Pediatrics, Sanford School of Medicine at the University of South Dakota, Vermillion, SD, United StatesBatten 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 proteins. We demonstrate substantial overlap in the protein interactomes of three transmembrane Batten proteins (CLN3, CLN6, and CLN8), and that their absence leads to synaptic depletion of key partners (i.e., SNAREs and tethers) and altered synaptic SNARE complexing in vivo, demonstrating a novel shared etiology.https://www.frontiersin.org/articles/10.3389/fnins.2022.834780/fulllysosomeneurodegenerationvesicle trafficBatten diseaseSNARE (soluble N-ethylmaleimide-sensitive fusion protein attachment protein receptor) |
| spellingShingle | Mitchell J. Rechtzigel Brandon L. Meyerink Brandon L. Meyerink Hannah Leppert Tyler B. Johnson Jacob T. Cain Gavin Ferrandino Danielle G. May Kyle J. Roux Kyle J. Roux Jon J. Brudvig Jon J. Brudvig Jill M. Weimer Jill M. Weimer Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment Frontiers in Neuroscience lysosome neurodegeneration vesicle traffic Batten disease SNARE (soluble N-ethylmaleimide-sensitive fusion protein attachment protein receptor) |
| title | Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment |
| title_full | Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment |
| title_fullStr | Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment |
| title_full_unstemmed | Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment |
| title_short | Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment |
| title_sort | transmembrane batten disease proteins interact with a shared network of vesicle sorting proteins impacting their synaptic enrichment |
| topic | lysosome neurodegeneration vesicle traffic Batten disease SNARE (soluble N-ethylmaleimide-sensitive fusion protein attachment protein receptor) |
| url | https://www.frontiersin.org/articles/10.3389/fnins.2022.834780/full |
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