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12 results on '"Ruth Singer"'

1. Treating late-onset Tay Sachs disease: Brain delivery with a dual trojan horse protein

Author

Esther Osher, Yossi Anis, Ruth Singer-Shapiro, Nataly Urshanski, Tamar Unger, Shira Albeck, Oren Bogin, Gary Weisinger, Fortune Kohen, Avi Valevski, Aviva Fattal-Valevski, Liora Sagi, Michal Weitman, Yulia Shenberger, Nadav Sagiv, Ruth Navon, Meir Wilchek, and Naftali Stern

Subjects
late-onset Tay-Sachs, β-hexosaminidase A, HexA, HEXA, HEXB, enzyme replacement therapy, Genetics, QH426-470, Cytology, QH573-671
Abstract

Tay-Sachs (TS) disease is a neurodegenerative disease resulting from mutations in the gene encoding the α-subunit (HEXA) of lysosomal β-hexosaminidase A (HexA). We report that (1) recombinant HEXA alone increased HexA activity and decreased GM2 content in human TS glial cells and peripheral mononuclear blood cells; 2) a recombinant chimeric protein composed of HEXA linked to two blood-brain barrier (BBB) entry elements, a transferrin receptor binding sequence and granulocyte-colony stimulating factor, associates with HEXB in vitro; reaches human cultured TS cells lysosomes and mouse brain cells, especially neurons, in vivo; lowers GM2 in cultured human TS cells; lowers whole brain GM2 concentration by approximately 40% within 6 weeks, when injected intravenously (IV) to adult TS-mutant mice mimicking the slow course of late-onset TS; and increases forelimbs grip strength. Hence, a chimeric protein equipped with dual BBB entry elements can transport a large protein such as HEXA to the brain, decrease the accumulation of GM2, and improve muscle strength, thereby providing potential treatment for late-onset TS.

Published
2024
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2. Language naming in Indigenous Australia: a view from western Arnhem Land

Author

Jill Vaughan, Ruth Singer, and Murray Garde

Subjects
Linguistics and Language, Communication, Language and Linguistics
Abstract

Language naming systems are local ways of organising diversity, yet the language names used by linguists are sometimes incommensurable with the lived social reality of speakers. The process of assigning language names is not neutral, trivial or objective: it is a highly political process driven and shaped by understandings of group identity, similarity and difference. Closer attention to local perspectives on language naming offers important insights into ideologies around social and linguistic differentiation. This paper draws together accounts of diverse language naming practices from across Indigenous Australia and applies a close lens to the region of western Arnhem Land. Through an examination of three groups (speakers of Bininj Kunwok, Mawng, and Burarra), we describe the range of strategies speakers use to divide up their local language ecologies, practices for naming lects, and the role of variation in the processes of differentiation. Naming practices between these groups show interesting similarities but also striking differences. We further highlight the interplay between two key processes which characterise local language naming strategies in the region: the ‘erasure’ of difference, typically from the perspective of a politically more powerful group, and the intentional creation of linguistic differentiation, or ausbau.

Published
2022

9. Grambank reveals the importance of genealogical constraints on linguistic diversity and highlights the impact of language loss

Author

Hedvig Skirgård, Hannah J. Haynie, Damián E. Blasi, Harald Hammarström, Jeremy Collins, Jay J. Latarche, Jakob Lesage, Tobias Weber, Alena Witzlack-Makarevich, Sam Passmore, Angela Chira, Luke Maurits, Russell Dinnage, Michael Dunn, Ger Reesink, Ruth Singer, Claire Bowern, Patience Epps, Jane Hill, Outi Vesakoski, Martine Robbeets, Noor Karolin Abbas, Daniel Auer, Nancy A. Bakker, Giulia Barbos, Robert D. Borges, Swintha Danielsen, Luise Dorenbusch, Ella Dorn, John Elliott, Giada Falcone, Jana Fischer, Yustinus Ghanggo Ate, Hannah Gibson, Hans-Philipp Göbel, Jemima A. Goodall, Victoria Gruner, Andrew Harvey, Rebekah Hayes, Leonard Heer, Roberto E. Herrera Miranda, Nataliia Hübler, Biu Huntington-Rainey, Jessica K. Ivani, Marilen Johns, Erika Just, Eri Kashima, Carolina Kipf, Janina V. Klingenberg, Nikita König, Aikaterina Koti, Richard G. A. Kowalik, Olga Krasnoukhova, Nora L. M. Lindvall, Mandy Lorenzen, Hannah Lutzenberger, Tânia R. A. Martins, Celia Mata German, Suzanne van der Meer, Jaime Montoya Samamé, Michael Müller, Saliha Muradoglu, Kelsey Neely, Johanna Nickel, Miina Norvik, Cheryl Akinyi Oluoch, Jesse Peacock, India O. C. Pearey, Naomi Peck, Stephanie Petit, Sören Pieper, Mariana Poblete, Daniel Prestipino, Linda Raabe, Amna Raja, Janis Reimringer, Sydney C. Rey, Julia Rizaew, Eloisa Ruppert, Kim K. Salmon, Jill Sammet, Rhiannon Schembri, Lars Schlabbach, Frederick W. P. Schmidt, Amalia Skilton, Wikaliler Daniel Smith, Hilário de Sousa, Kristin Sverredal, Daniel Valle, Javier Vera, Judith Voß, Tim Witte, Henry Wu, Stephanie Yam, Jingting Ye, Maisie Yong, Tessa Yuditha, Roberto Zariquiey, Robert Forkel, Nicholas Evans, Stephen C. Levinson, Martin Haspelmath, Simon J. Greenhill, Quentin D. Atkinson, Russell D. Gray, Skirgård, Hedvig [0000-0002-7748-2381], Haynie, Hannah J [0000-0003-2237-230X], Blasi, Damián E [0000-0002-9885-1414], Latarche, Jay J [0000-0002-0486-248X], Lesage, Jakob [0000-0002-3751-9637], Witzlack-Makarevich, Alena [0000-0003-0138-4635], Chira, Angela [0000-0002-2964-7583], Maurits, Luke [0000-0002-3130-4256], Dinnage, Russell [0000-0003-0846-2819], Dunn, Michael [0000-0001-5349-5252], Reesink, Ger [0000-0002-8724-7887], Singer, Ruth [0000-0003-4915-3262], Bowern, Claire [0000-0002-9512-4393], Epps, Patience [0000-0002-7429-7885], Hill, Jane [0000-0002-4501-1016], Vesakoski, Outi [0000-0002-7220-3347], Abbas, Noor Karolin [0000-0002-6602-4772], Borges, Robert D [0000-0002-7647-4048], Dorenbusch, Luise [0000-0003-0410-6921], Dorn, Ella [0000-0002-6344-4907], Fischer, Jana [0000-0003-0576-1098], Ghanggo Ate, Yustinus [0000-0003-4378-5277], Gibson, Hannah [0000-0003-2324-3147], Goodall, Jemima A [0000-0002-0310-9445], Gruner, Victoria [0000-0002-4504-1274], Harvey, Andrew [0000-0003-3659-4704], Hayes, Rebekah [0000-0003-0978-2646], Heer, Leonard [0000-0002-1569-9761], Hübler, Nataliia [0000-0002-0013-563X], Just, Erika [0000-0001-7244-3714], Kashima, Eri [0000-0002-7534-7047], König, Nikita [0000-0001-9582-9361], Koti, Aikaterina [0000-0002-8155-7120], Kowalik, Richard GA [0000-0003-4903-997X], Krasnoukhova, Olga [0000-0002-1953-7954], Lindvall, Nora LM [0000-0003-4640-7589], Lorenzen, Mandy [0000-0001-5433-8224], Lutzenberger, Hannah [0000-0003-0574-4060], Martins, Tânia RA [0000-0002-3648-2190], van der Meer, Suzanne [0000-0002-4232-8675], Montoya Samamé, Jaime [0000-0003-3797-1161], Müller, Michael [0000-0002-2027-7301], Norvik, Miina [0000-0001-5781-3916], Oluoch, Cheryl Akinyi [0000-0003-2465-719X], Peck, Naomi [0000-0003-3567-813X], Poblete, Mariana [0000-0003-0253-712X], Raabe, Linda [0000-0002-3684-3742], Reimringer, Janis [0000-0001-6769-8907], Rey, Sydney C [0000-0002-5657-9221], Rizaew, Julia [0000-0002-4250-8035], Salmon, Kim K [0000-0001-8515-1008], Sammet, Jill [0000-0003-0869-795X], Schmidt, Frederick WP [0000-0002-1429-966X], Skilton, Amalia [0000-0002-9848-5688], de Sousa, Hilário [0000-0002-3790-8245], Vera, Javier [0000-0002-5234-6279], Wu, Henry [0000-0002-5762-3124], Ye, Jingting [0000-0001-8813-131X], Yuditha, Tessa [0000-0002-1280-7245], Zariquiey, Roberto [0000-0002-1421-1314], Forkel, Robert [0000-0003-1081-086X], Evans, Nicholas [0000-0003-0893-3713], Levinson, Stephen C [0000-0001-8961-5316], Greenhill, Simon J [0000-0001-7832-6156], Atkinson, Quentin D [0000-0002-8499-7535], Gray, Russell D [0000-0002-9858-0191], and Apollo - University of Cambridge Repository

Subjects
Multidisciplinary, Cognition, Databases, Factual, Humans, Linguistics, Language
Abstract

While global patterns of human genetic diversity are increasingly well characterized, the diversity of human languages remains less systematically described. Here we outline the Grambank database. With over 400,000 data points and 2,400 languages, Grambank is the largest comparative grammatical database available. The comprehensiveness of Grambank allows us to quantify the relative effects of genealogical inheritance and geographic proximity on the structural diversity of the world's languages, evaluate constraints on linguistic diversity, and identify the world's most unusual languages. An analysis of the consequences of language loss reveals that the reduction in diversity will be strikingly uneven across the major linguistic regions of the world. Without sustained efforts to document and revitalize endangered languages, our linguistic window into human history, cognition and culture will be seriously fragmented. Genealogy versus geography Constraints on grammar Unusual languages Language loss Conclusion

Published
2023

12. Indigenous Multilingualism at Warruwi : Cultivating Linguistic Diversity in an Australian Community

Author

Ruth Singer and Ruth Singer

Subjects
Multilingualism--Political aspects--Australia--Arnhem Land (N.T.), Language and culture--Australia--Arnhem Land (N.T.), Multilingualism--Social aspects--Australia--Arnhem Land (N.T.), Anthropological linguistics--Australia--Arnhem Land (N.T.), Maung (Australian people)--Languages
Abstract

This book is an exploration of the role of language at Warruwi Community, a remote Indigenous settlement in northern Australia. It explores how language use and people's ideas about language are embedded in contemporary Indigenous life there.Using an ethnographic approach, the book examines what language at Warruwi means in the context of the history of the community, ongoing social and political changes and the continuing importance of ancestral traditions. Children growing up at Warruwi still learn to speak many small Indigenous languages. This is remarkable not just in the Australian context, where many Indigenous languages are no longer spoken, but around the world as this kind of multilingualism in small languages persists only in a few remaining pockets. The way that people use many languages in their daily life at Warruwi reveals how high levels of linguistic diversity can be maintained in a small community.This detailed study of the creation of linguistic diversity is relevant to sociolinguistics, linguistic typology, historical linguistics and evolutionary linguistics. More generally, this book is for linguists, anthropologists and anyone with an interest in contemporary Australian Indigenous lives.

Published
2023

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