Publications

Abstract

Spinocerebellar ataxia type 14 (SCA14) is a subtype of the autosomal dominant cerebellar ataxias that is characterized by slowly progressive cerebellar dysfunction and neurodegeneration. SCA14 is caused by mutations in the PRKCG gene, encoding protein kinase C gamma (PKCγ). Despite the identification of 40 distinct disease-causing mutations in PRKCG, the pathological mechanisms underlying SCA14 remain poorly understood. Here we report the molecular neuropathology of SCA14 in post-mortem cerebellum and in human patient-derived induced pluripotent stem cells (iPSCs) carrying two distinct SCA14 mutations in the C1 domain of PKCγ, H36R and H101Q. We show that endogenous expression of these mutations results in the cytoplasmic mislocalization and aggregation of PKCγ in both patient iPSCs and cerebellum. PKCγ aggregates were not efficiently targeted for degradation. Moreover, mutant PKCγ was found to be hyper-activated, resulting in increased substrate phosphorylation. Together, our findings demonstrate that a combination of both, loss-of-function and gain-of-function mechanisms are likely to underlie the pathogenesis of SCA14, caused by mutations in the C1 domain of PKCγ. Importantly, SCA14 patient iPSCs were found to accurately recapitulate pathological features observed in post-mortem SCA14 cerebellum, underscoring their potential as relevant disease models and their promise as future drug discovery tools.

Abstract

The ISOcat Data Category Registry provides a community computing environment for creating, storing, retrieving, harmonizing and standardizing data category specifications (DCs), used to register linguistic terms used in various fields. This chapter recounts the history of DC documentation in TC 37, beginning from paper-based lists created for lexicographers and terminologists and progressing to the development of a web-based resource for a much broader range of users. While describing the considerable strides that have been made to collect a very large comprehensive collection of DCs, it also outlines difficulties that have arisen in developing a fully operative web-based computing environment for achieving consensus on data category names, definitions, and selections and describes efforts to overcome some of the present shortcomings and to establish positive working procedures designed to engage a wide range of people involved in the creation of language resources.

Abstract

Im ISOcat-Datenkategorie-Register (Data Category Registry, www.isocat.org) des Technischen Komitees ISO/TC 37 (Terminology and other language and content resources) werden Feldnamen und Werte für Sprachressourcen beschrieben. Empfohlene Feldnamen und zuverlässige Definitionen sollen dazu beitragen, dass Sprachdaten unabhängig von Anwendungen, Plattformen und Communities of Practice (CoP) wiederverwendet werden können. Datenkategorie-Gruppen (Data Category Selections) können eingesehen, ausgedruckt, exportiert und nach kostenloser Registrierung auch neu erstellt werden.

Abstract

In the present study, event-related brain potentials (ERP) were recorded to investigate the role of pitch accent and lexical tone in spoken discourse comprehension. Chinese was used as material to explore the potential difference in the nature and time course of brain responses to sentence meaning as indicated by pitch accent and to lexical meaning as indicated by tone. In both cases, the pitch contour of critical words was varied. The results showed that both inconsistent pitch accent and inconsistent lexical tone yielded N400 effects, and there was no interaction between them. The negativity evoked by inconsistent pitch accent had the some topography as that evoked by inconsistent lexical tone violation, with a maximum over central–parietal electrodes. Furthermore, the effect for the combined violations was the sum of effects for pure pitch accent and pure lexical tone violation. However, the effect for the lexical tone violation appeared approximately 90 ms earlier than the effect of the pitch accent violation. It is suggested that there might be a correspondence between the neural mechanism underlying pitch accent and lexical meaning processing in context. They both reflect the integration of the current information into a discourse context, independent of whether the current information was sentence meaning indicated by accentuation, or lexical meaning indicated by tone. In addition, lexical meaning was processed earlier than sentence meaning conveyed by pitch accent during spoken language processing.

Abstract

Electroencephalography (EEG) provides high temporal resolution cognitive information from non-invasive recordings. However, one of the common practices-using a subset of sensors in ERP analysis is hard to provide a holistic and precise dynamic results. Selecting or grouping subsets of sensors may also be subject to selection bias, multiple comparison, and further complicated by individual differences in the group-level analysis. More importantly, changes in neural generators and variations in response magnitude from the same neural sources are difficult to separate, which limit the capacity of testing different aspects of cognitive hypotheses. We introduce EasyEEG, a toolbox that includes several multivariate analysis methods to directly test cognitive hypotheses based on topographic responses that include data from all sensors. These multivariate methods can investigate effects in the dimensions of response magnitude and topographic patterns separately using data in the sensor space, therefore enable assessing neural response dynamics. The concise workflow and the modular design provide user-friendly and programmer-friendly features. Users of all levels can benefit from the open-sourced, free EasyEEG to obtain a straightforward solution for efficient processing of EEG data and a complete pipeline from raw data to final results for publication.

Abstract

L'A. étudie les corrélations entre structure syntaxique et fonction pragmatique dans les alternances de voix en akatek, une langue maya appartenant au sous-groupe Q'anjob'ala. Les alternances pragmatiques de voix sont les mécanismes par lesquels les langues encodent les différents degrés de topicalité des deux principaux participants d'un événement sémantiquement transitif, l'agent et le patient. A l'aide d'une analyse quantitative, l'A. évalue la topicalité de ces participants et identifie les structures syntaxiques permettant d'exprimer les quatre principales fonctions de voix en akatek : active-directe, inverse, passive et antipassive

Abstract

This article presents data on cardinal numerals in three sign languages from small-scale communities with hereditary deafness. The unusual features found in these data considerably extend the known range of typological variety across sign languages. Some features, such as non-decimal numeral bases, are unattested in sign languages, but familiar from spoken languages, while others, such as subtractive sub-systems, are rare in sign and speech. We conclude that for a complete typological appraisal of a domain, an approach to cross-modal typology, which includes a typologically diverse range of sign languages in addition to spoken languages, is both instructive and feasible.

Abstract

Although bilingual speakers are very good at selectively using one language rather than another, sometimes language selection errors occur. To investigate how bilinguals monitor their speech errors and control their languages in use, we recorded event-related potentials (ERPs) in unbalanced Dutch-English bilingual speakers in a cued language-switching task. We tested the conflict-based monitoring model of Nozari and colleagues by investigating the error-related negativity (ERN) and comparing the effects of the two switching directions (i.e., to the first language, L1 vs. to the second language, L2). Results show that the speakers made more language selection errors when switching from their L2 to the L1 than vice versa. In the EEG, we observed a robust ERN effect following language selection errors compared to correct responses, reflecting monitoring of speech errors. Most interestingly, the ERN effect was enlarged when the speakers were switching to their L2 (less conflict) compared to switching to the L1 (more conflict). Our findings do not support the conflict-based monitoring model. We discuss an alternative account in terms of error prediction and reinforcement learning.

Abstract

Although bilingual speakers are very good at selectively using one language rather than another, sometimes language selection errors occur. We examined the relative contribution of top-down cognitive control and bottom-up language priming to these errors. Unbalanced Dutch-English bilinguals named pictures and were cued to switch between languages under time pressure. We also manipulated the number of same-language trials before a switch (long vs. short runs). Results show that speakers made more language selection errors when switching from their second language (L2) to the first language (L1) than vice versa. Furthermore, they made more errors when switching to the L1 after a short compared to a long run of L2 trials. In the reverse switching direction (L1 to L2), run length had no effect. These findings are most compatible with an account of language selection errors that assigns a strong role to top-down processes of cognitive control.

Abstract

We describe ViCoS, a tool for constructing and visualising conceptual spaces in the area of language documentation. ViCoS allows users to enrich existing lexical information about the words of a language with conceptual knowledge. Their work towards language-based, informal ontology building must be supported by easy-to-use workflows and supporting software, which we will demonstrate.

Abstract

It is undisputed that presenting a rhythmic stimulus leads to a measurable brain response that follows the rhythmic structure of this stimulus. What is still debated, however, is the question whether this brain response exclusively reflects a regular repetition of evoked responses, or whether it also includes entrained oscillatory activity. Here we systematically present evidence in favor of an involvement of entrained neural oscillations in the processing of rhythmic input while critically pointing out which questions still need to be addressed before this evidence could be considered conclusive. In this context, we also explicitly discuss the potential functional role of such entrained oscillations, suggesting that these stimulus-aligned oscillations reflect, and serve as, predictive processes, an idea often only implicitly assumed in the literature.

Abstract

Psycholinguistic research has typically portrayed speech production as a relatively automatic process. This is because when errors are made, they occur as seldom as one in every thousand words we utter. However, it has long been recognised that we need some form of control over what we are currently saying and what we plan to say. This capacity to both monitor our inner speech and self-correct our speech output has often been assumed to be a property of the language comprehension system. More recently, it has been demonstrated that speech production benefits from interfacing with more general cognitive processes such as selective attention, short-term memory (STM) and online response monitoring to resolve potential conflict and successfully produce the output of a verbal plan. The conditions and levels of representation according to which these more general planning, monitoring and control processes are engaged during speech production remain poorly understood. Moreover, there remains a paucity of information about their neural substrates, despite some of the first evidence of more general monitoring having come from electrophysiological studies of error related negativities (ERNs). While aphasic speech errors continue to be a rich source of information, there has been comparatively little research focus on instances of speech repair. The purpose of this Frontiers Research Topic is to provide a forum for researchers to contribute investigations employing behavioural, neuropsychological, electrophysiological, neuroimaging and virtual lesioning techniques. In addition, while the focus of the research topic is on novel findings, we welcome submission of computational simulations, review articles and methods papers.

Abstract

This paper discusses the construction of a cross-linguistic, bimodal corpus containing three modes of expression: expressions from two sign languages, speech and gestural expressions in two spoken languages and pantomimic expressions by users of two spoken languages who are requested to convey information without speaking. We discuss some problems and tentative solutions for the annotation of utterances expressing spatial information about referents in these three modes, suggesting a set of comparable codes for the description of both sign and gesture. Furthermore, we discuss the processing of entered annotations in ELAN, e.g. relating descriptive annotations to analytic annotations in all three modes and performing relational searches across annotations on different tiers.

Abstract

The lexicons of many sign languages hold large proportions of “frozen” forms, viz. signs that are generally considered to have been formed productively (as classifier predicates), but that have diachronically undergone processes of lexicalisation. Nederlandse Gebarentaal (Sign Language of the Netherlands; henceforth: NGT) also has many of these signs (Van der Kooij 2002, Zwitserlood 2003). In contrast to the general view on “frozen” forms, a few researchers claim that these signs may be formed according to productive sign formation rules, notably Brennan (1990) for BSL, and Meir (2001, 2002) for ISL. Following these claims, I suggest an analysis of “frozen” NGT signs as morphologically complex, using the framework of Distributed Morphology. The signs in question are derived in a similar way as classifier predicates; hence their similar form (but diverging characteristics). I will indicate how and why the structure and use of classifier predicates and “frozen” forms differ. Although my analysis focuses on NGT, it may also be applicable to other sign languages.

Abstract

This paper reports on an exploration of the ways in which multiple entities are expressed in Turkish Sign Language (TİD). The (descriptive and quantitative) analyses provided are based on a corpus of both spontaneous data and specifically elicited data, in order to provide as comprehensive an account as possible. We have found several devices in TİD for expression of multiple entities, in particular localization, spatial plural predicate inflection, and a specific form used to express multiple entities that are side by side in the same configuration (not reported for any other sign language to date), as well as numerals and quantifiers. In contrast to some other signed languages, TİD does not appear to have a productive system of plural reduplication. We argue that none of the devices encountered in the TİD data is a genuine plural marking device and that the plural interpretation of multiple entity localizations and plural predicate inflections is a by-product of the use of space to indicate the existence or the involvement in an event of multiple entities.