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 terminology Community of Practice has long standardized data categories in the framework of ISO TC 37. ISO 12620:2009 specifies the data model and procedures for a Data Category Registry (DCR), which has been implemented by the Max Planck Institute for Psycholinguistics as the ISOcat DCR. The DCR has been used by not only ISO TC 37, but also by the CLARIN research infra-structure. This paper describes how the needs of these communities have started to diverge and the process of segueing from a DCR to a Data Concept Registry in order to meet the needs of both communities.

Abstract

Set is widely used as a kind of basic data structure. However, when it is used for large scale data set the cost of storage, search and transport is overhead. The bloom filter uses a fixed size bit string to represent elements in a static set, which can reduce storage space and search cost that is a fixed constant. The time-space efficiency is achieved at the cost of a small probability of false positive in membership query. However, for many applications the space savings and locating time constantly outweigh this drawback. Dynamic bloom filter (DBF) can support concisely representation and approximate membership queries of dynamic set instead of static set. It has been proved that DBF not only possess the advantage of standard bloom filter, but also has better features when dealing with dynamic set. This paper proposes a time-dependent multiple bit-strings bloom filter (TMBF) which roots in the DBF and targets on dynamic incremental set. TMBF uses multiple bit-strings in time order to present a dynamic increasing set and uses backward searching to test whether an element is in a set. Based on the system logs from a real P2P file sharing system, the evaluation shows a 20% reduction in searching cost compared to DBF.

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

This study investigates how Mandarin Chinese speaking children and adults use prosody to mark focus in spontaneous speech. SVO sentences were elicited from 4- and 8-year-olds and adults in a game setting. Sentence-medial verbs were acoustically analysed for both duration and pitch range in different focus conditions. We have found that like the adults, the 8-year-olds used both duration and pitch range to distinguish focus from non-focus. The 4-year-olds used only duration to distinguish focus from non-focus, unlike the adults and 8-year-olds. None of the three groups of speakers distinguished contrastive focus from non-contrastive focus using pitch range or duration. Regarding the distinction between narrow focus from broad focus, the 4- and 8-year-olds used both pitch range and duration for this purpose, while the adults used only duration

Abstract

This study investigates how Mandarin Chinese speaking children use prosody to distinguish focus from non-focus, and focus types differing in size of constituent and contrastivity. SVO sentences were elicited from four- and eight-year-olds in a game setting. Sentence-medial verbs were acoustically analysed for both duration and pitch range in different focus conditions. The children started to use duration to differentiate focus from non-focus at the age of four. But their use of pitch range varied with age and depended on non-focus conditions (pre- vs. postfocus) and the lexical tones of the verbs. Further, the children in both age groups used pitch range but not duration to differentiate narrow focus from broad focus, and they did not differentiate contrastive narrow focus from non-contrastive narrow focus using duration or pitch range. The results indicated that Chinese children acquire the prosodic means (duration and pitch range) of marking focus in stages, and their acquisition of these two means appear to be early, compared to children speaking an intonation language, for example, Dutch.

Abstract

Neural structures change with age but there is no consensus on the exact processes involved. This study tested the hypothesis that white and grey matter in the language network changes during aging according to a “last in, first out” process. The fractional anisotropy (FA) of white matter and cortical thickness of grey matter were measured in 36 participants whose ages ranged from 55 to 79 years. Within the language network, the dorsal pathway connecting the mid-to-posterior superior temporal cortex (STC) and the inferior frontal cortex (IFC) was affected more by aging in both FA and thickness than the other dorsal pathway connecting the STC with the premotor cortex and the ventral pathway connecting the mid-to-anterior STC with the ventral IFC. These results were independently validated in a second group of 20 participants whose ages ranged from 50 to 73 years. The pathway that is most affected during aging matures later than the other two pathways (which are present at birth). The results are interpreted as showing that the neural structures which mature later are affected more than those that mature earlier, supporting the “last in, first out” theory.

Abstract

This paper presents VarClass, an open-source tool for language identification available both to be downloaded as well as through a graphical user-friendly interface. The main difference of VarClass in comparison to other state-of-the-art language identification tools is its focus on language varieties. General purpose language identification tools do not take language varieties into account and our work aims to fill this gap. VarClass currently contains language models for over 27 languages in which 10 of them are language varieties. We report an average performance of over 90.5% accuracy in a challenging dataset. More language models will be included in the upcoming months

Abstract

This article provides a first overview of some striking grammatical structures in Türk Idotscedilaret Dili (Turkish Sign Language, TID), the sign language used by the Deaf community in Turkey. The data are described with a typological perspective in mind, focusing on aspects of TID grammar that are typologically unusual across sign languages. After giving an overview of the historical, sociolinguistic and educational background of TID and the language community using this sign language, five domains of TID grammar are investigated in detail. These include a movement derivation signalling completive aspect, three types of nonmanual negation — headshake, backward head tilt, and puffed cheeks — and their distribution, cliticization of the negator NOT to a preceding predicate host sign, an honorific whole-entity classifier used to refer to humans, and a question particle, its history and current status in the language. A final evaluation points out the significance of these data for sign language research and looks at perspectives for a deeper understanding of the language and its history.

Abstract

Indo-Pakistani Sign Language (IPSL) is the sign language used by deaf communities in a large region across India and Pakistan. This visual-gestural language has a dedicated construction for specifically expressing reciprocal relationships, which can be applied to agreement verbs and to auxiliaries. The reciprocal construction relies on a change in the movement pattern of the signs it applies to. In addition, IPSL has a number of other strategies which can have a reciprocal interpretation, and the IPSL lexicon includes a good number of inherently reciprocal signs. All reciprocal expressions can be modified in complex ways that rely on the grammatical use of the sign space. Considering grammaticalisation and lexicalisation processes linking some of these constructions is also important for a better understanding of reciprocity in IPSL.

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

The present study investigates how long after adoption adoptees forget the phonology of their birth language. Chinese children who were adopted by Dutch families were tested on the perception of birth language tone contrasts before, during, and after perceptual training. Experiment 1 investigated Cantonese tone 2 (High-Rising) and tone 5 (Low-Rising), and Experiment 2 investigated Mandarin tone 2 (High-Rising) and tone 3 (Low-Dipping). In both experiments, participants were adoptees and non-adopted Dutch controls. Results of both experiments show that the tone contrasts were very difficult to perceive for the adoptees, and that adoptees were not better at perceiving the tone contrasts than their non-adopted Dutch peers, before or after training. This demonstrates that forgetting took place relatively soon after adoption, and that the re-exposure that the adoptees were presented with did not lead to an improvement greater than that of the Dutch control participants. Thus, the findings confirm what has been anecdotally reported by adoptees and their parents, but what had not been empirically tested before, namely that birth language forgetting occurs very soon after adoption

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

For most people, speech production is relatively effortless and error-free. Yet it has long been recognized that we need some type of control over what we are currently saying and what we plan to say. Precisely how we monitor our internal and external speech has been a topic of research interest for several decades. The predominant approach in psycholinguistics has assumed monitoring of both is accomplished via systems responsible for comprehending others' speech.

This special topic aimed to broaden the field, firstly by examining proposals that speech production might also engage more general systems, such as those involved in action monitoring. A second aim was to examine proposals for a production-specific, internal monitor. Both aims require that we also specify the nature of the representations subject to monitoring.

Abstract

Given the limited processing capabilities of the sensory system, it is essential that attended information is gated to downstream areas, whereas unattended information is blocked. While it has been proposed that alpha band (8–13 Hz) activity serves to route information to downstream regions by inhibiting neuronal processing in task-irrelevant regions, this hypothesis remains untested. Here we investigate how neuronal oscillations detected by electroencephalography in visual areas during working memory encoding serve to gate information reflected in the simultaneously recorded blood-oxygenation-level-dependent (BOLD) signals recorded by functional magnetic resonance imaging in downstream ventral regions. We used a paradigm in which 16 participants were presented with faces and landscapes in the right and left hemifields; one hemifield was attended and the other unattended. We observed that decreased alpha power contralateral to the attended object predicted the BOLD signal representing the attended object in ventral object-selective regions. Furthermore, increased alpha power ipsilateral to the attended object predicted a decrease in the BOLD signal representing the unattended object. We also found that the BOLD signal in the dorsal attention network inversely correlated with visual alpha power. This is the first demonstration, to our knowledge, that oscillations in the alpha band are implicated in the gating of information from the visual cortex to the ventral stream, as reflected in the representationally specific BOLD signal. This link of sensory alpha to downstream activity provides a neurophysiological substrate for the mechanism of selective attention during stimulus processing, which not only boosts the attended information but also suppresses distraction. Although previous studies have shown a relation between the BOLD signal from the dorsal attention network and the alpha band at rest, we demonstrate such a relation during a visuospatial task, indicating that the dorsal attention network exercises top-down control of visual alpha activity.

Abstract

This study investigates the morphological and morphosyntactic characteristics of hand configurations in signs, particularly in Nederlandse Gebarentaal (NGT). The literature on sign languages in general acknowledges that hand configurations can function as morphemes, more specifically as classifiers , in a subset of signs: verbs expressing the motion, location, and existence of referents (VELMs). These verbs are considered the output of productive sign formation processes. In contrast, other signs in which similar hand configurations appear ( iconic or motivated signs) have been considered to be lexicalized signs, not involving productive processes. This research report shows that meaningful hand configurations have (at least) two very different functions in the grammar of NGT (and presumably in other sign languages, too). First, they are agreement markers on VELMs, and hence are functional elements. Second, they are roots in motivated signs, and thus lexical elements. The latter signs are analysed as root compounds and are formed from various roots by productive processes. The similarities in surface form and differences in morphosyntactic characteristics observed in comparison of VELMs and root compounds are attributed to their different structures and to the sign language interface between grammar and phonetic form

Abstract

Review: User friendliness of the first dictionary of Sign Language of the Netherlands can be improved

Abstract

(Needed: staff for residential care home with good eye-hand coordination. Measuring NGT-skills.)

Abstract

The Corpus NGT

Abstract

(The Corpus NGT and the daily practice of language teaching)

Abstract

(The Corpus NGT and teacher NGT/interpreter NGT training)

Abstract

Although in many respects sign languages have a similar structure to that of spoken languages, the different modalities in which both types of languages are expressed cause differences in structure as well. One of the most striking differences between spoken and sign languages is the influence of the interface between grammar and PF on the surface form of utterances. Spoken language words and phrases are in general characterized by sequential strings of sounds, morphemes and words, while in sign languages we find that many phonemes, morphemes, and even words are expressed simultaneously. A linguistic model should be able to account for the structures that occur in both spoken and sign languages. In this paper, I will discuss the morphological/ morphosyntactic structure of signs in Nederlandse Gebarentaal (Sign Language of the Netherlands, henceforth NGT), with special focus on the components ‘place of articulation’ and ‘handshape’. I will focus on their multiple functions in the grammar of NGT and argue that the framework of Distributed Morphology (DM), which accounts for word formation in spoken languages, is also suited to account for the formation of structures in sign languages. First I will introduce the phonological and morphological structure of NGT signs. Then, I will briefly outline the major characteristics of the DM framework. Finally, I will account for signs that have the same surface form but have a different morphological structure by means of that framework.