Canavan disease: a white matter disorder

Breakdown of oligodendrocyte-neuron interactions in white matter (WM), such as the loss of myelin, results in axonal dysfunction and hence a disruption of information processing between brain regions. The major feature of leukodystrophies is the lack of proper myelin formation during early development or the onset of myelin loss late in life. These early childhood WM diseases are described as hypomyelination or dysmyelination arising from a primary block in normal myelin synthesis because of a genetic mutation expressed in oligodendrocytes, or failure in myelination secondary to neuronal or astroglial dysfunctions (van der Knaap 2001 Dev. Med. Child Neurol. 43:705-712). Here, we describe the pathophysiological parameters of Canavan disease (CD), caused by genetic mutations of the aspartoacylase (ASPA) gene, a metabolic enzyme restricted in the central nervous system (CNS) to oligodendrocytes. CD presents pathophysiological dysfunctions similar to diseases caused by myelin gene mutations, such as Pelizaeus-Merzbacher disease (PMD) and several animal models, such as myelin deficient rat (md), jimpy (jp), shiverer (sh), and quaking (qk viable) mutant mice. These single gene mutations have pleiotropic effects, whereby the alteration of one myelin gene expression disrupts functional expression of other oligodendrocyte genes with an outcome of hypomyelination/dysmyelination. Among all of the known leukodystrophies, CD is the first disorder, which was approved and tested for the adeno-associated virus vector (AAV)-ASPA gene therapy (Leone et al. 2000 Ann. Neurol. 48:27-38; Janson et al. 2001 Trends Neurosci. 24:706-712) without much success following the first two attempts. ASPA gene delivery attempts in animal models have shown a lowering of N-acetyl L-aspartate and a change in motor functions, while sponginess of the WM, a characteristic of CD remained unchanged (Matalon et al. 2003 Mol. Ther. 7 (5, Part 1):580-587; McPhee et al. 2005 Brain Res. Mol. Brain Res. 135:112-121) even with better viral serotype and delivery of the gene during early phase of development (Klugmann et al. 2005 Mol. Ther. 11:745-753). While different approaches are being sought for the success of gene therapy, there are pivotal developmental questions to address regarding the specific regions of the CNS and cell lineages that become the target for the onset and progression of CD symptoms from early to late stages of development.     

The role of vision in the control of continuous multijoint movements

The authors investigated whether visual fixations during a continuous graphical task were related to arm endpoint kinematics, joint motions, or joint control. The pattern of visual fixations across various shapes and the relationship between temporal and spatial events of the moving limb and visual fixations were assessed. Participants (N=16) performed movements of varying shapes by rotating the shoulder and elbow joints in the transverse plane at a comfortable pace. Across shapes, eye movements consisted of a series of fixations, with the eyes leading the hand. Fixations were spatially related to modulation of joint motion and were temporally related to the portions of the movement where curvature was the highest. Gathering of information related to modulation of interactive torques arising from passive forces from movement of a linked system occurred when the velocity of the movement (a) was the lowest and (b) was ahead of the moving limb, suggesting that that information is used in a feedforward manner.     

Enhancing Creativity With Combined Transcranial Direct Current and Random Noise Stimulation of the Left Dorsolateral Prefrontal Cortex and Inferior Frontal Gyrus

Creativity is a fundamental human accomplishment from scientific advances to composing music. The left dorsolateral prefrontal cortex (DLPFC) and inferior frontal gyrus (IFG) are important metacontrol hubs in flexibility and persistence brain states, respectively. Those hubs are related to divergent thinking, insight problem-solving, and convergent thinking. In this double-blind, between-subjects study, 81 healthy participants were randomly assigned to one of three groups (n = 27) that received a combined transcranial direct current stimulation–transcranial random noise stimulation (tDCS-tRNS) protocol with the anode over the left DLPFC and cathode over the left IFG (+DLPFC−IFG), the opposite montage (−DLPFC+IFG), and a sham group (+DLPFC−IFG). Both active tDCS-tRNS groups received 20 min of 1 mA tDCS with 1 mA (100–500 Hz) tRNS. Creativity was assessed before (baseline) and during stimulation with the Unusual Uses, Picture Completion (PC), Remote Association test (RAT), Matchstick Arithmetic (MA), and Nine-dot (ND) problems. Only the +DLPFC−IFG group had significantly higher scores compared with sham in the RAT (p = .009), PC fluency (p = .018), PC originality (p = .007), ND (p = .007), and MA (p = .032). Overall, −DLPFC+IFG had greater scores in all creativity tests compared with sham. Implications from the metacontrol theory are discussed.     

The role of mindfulness and compassion in early adults' subsequent mental health,coping and compliance with health guidelines during the COVID-19 pandemic: A prospective longitudinal study

The present study explored prospective links between trait mindfulness and compassion on subsequent coping and compliance with Centers for Disease Control (CDC) guidelines and indirect effects via well-being and internalized distress during the COVID-19 pandemic. The study included N = 736 US college students who participated in a three-wave longitudinal study across a single academic year. The first two assessment waves took place in 2018 and 2019, respectively, while the third wave took place in May 2020 during the COVID-19 pandemic. Participants completed self-report measures of trait mindfulness, compassion, well-being, internalized distress, coping, and compliance with CDC health guidelines. Results of a series of autoregressive, cross-lagged panel models revealed that trait mindfulness was associated with better coping via indirect effects of greater well-being and lower internalized distress. Greater compassion was linked with greater adherence to CDC guidelines. Findings suggest that trait mindfulness and compassion may play a role in college students' coping and compliance during the pandemic.     

The European University at St. Petersburg: a case study in sociology of post-Soviet knowledge

The article presents results of an ongoing study of centers of intellectual innovations in post-Soviet Russia. Using the European University at St. Petersburg as the main object of their analysis, the authors demonstrate how new models of academic careers, which became available in the 1980s and 1990s, were eventually institutionalized as new models of knowledge production and educational practices. Supported by American foundations, this private university had to invent a new institutional structure and to position itself within the field of higher education, still mostly dominated by the state.     

Angiotensin-(1-7)/Mas axis integrity is required for the expression of object recognition memory

It has been shown that the brain has its own intrinsic renin-angiotensin system (RAS) and angiotensin-(1-7) (Ang-(1-7)) is particularly interesting, because it appears to counterbalance most of the Ang II effects. Ang-(1-7) exerts its biological function through activation of the G-protein-coupled receptor Mas. Interestingly, hippocampus is one of the regions with higher expression of Mas. However, the role of Ang-(1-7)/Mas axis in hippocampus-dependent memories is still poorly understood. Here we demonstrated that Mas ablation, as well as the blockade of Mas in the CA1-hippocampus, impaired object recognition memory (ORM). We also demonstrated that the blockade of Ang II receptors AT1, but not AT2, recovers ORM impairment of Mas-deficient mice. Considering that high concentrations of Ang-(1-7) may activate AT1 receptors, nonspecifically, we evaluate the levels of Ang-(1-7) and its main precursors Ang I and Ang II in the hippocampus of Mas-deficient mice. The Ang I and Ang II levels are unaltered in the whole hipocampus of MasKo. However, Ang-(1-7) concentration is increased in the whole hippocampus of MasKo mice, as well as in the CA1 area. Taken together, our findings suggest that the functionality of the Ang-(1-7)/Mas axis is essential for normal ORM processing.     

Differential diagnosis between apraxia and dysarthria based on acoustic analysis

Acoustic analysis provides objective quantitative measures of speech that enable a comprehensive and accurate understanding of motor disorders and complement the traditional measures. This paper aims to distinguish between normal and pathological speech, more specifically between apraxia of speech and spastic dysarthria in native Spanish speaking patients using acoustic parameters. Participants (4 aphasic with apraxia of speech, 4 with spastic dysarthria, and 15 without speech disorders) performed three different tasks: repeating the syllable sequence [pa-ta-ka], repeating the isolated syllable [pa] and repeating the vowel sequence [i-u]. The results showed that the normative values of motor control, in general, coincide with those obtained in previous research on native English speakers. They also show that damage to motor control processes results in a decrease in the rate of alternating and sequential movements and an increase in the inter-syllabic time for both types of movements. A subset of the acoustic parameters analyzed, those that measure motor planning processes, enable differentiation between normal population and apraxic and dysarthric patients, and between the latter. The differences between the pathological groups support the distinction between motor planning and motor programming as described by van der Merwe's model of sensorimotor processing (1997).     

Paternal influences on infant temperament: Effects of father internalizing problems,parenting-related stress,and temperament

Temperament ratings were obtained from 98 fathers when their infants were 4 and 6 months of age to examine effects of paternal characteristics on infant temperament. Parental stress, internalizing symptoms, and father's temperament were considered as factors possibly contributing to differences in their child's temperament.     

The bilingual L2 advantage in recognition memory

To better understand the mechanisms by which bilingual proficiency impacts memory processes, two recognition memory experiments were conducted with matched monolingual and bilingual samples. In Experiment 1, monolingual speakers of English and Spanish studied high- and low-frequency words under full attention or cognitive load conditions. In Experiment 2, Spanish–English bilingual participants studied high- and low-frequency words under full-attention conditions in each language. For both monolinguals and bilinguals, low-frequency words were better recognized than high-frequency words. The central new findings were that bilingual recognition was more accurate in the less fluent language (L2) than in the more fluent language (L1) and that bilingual L2 recognition was more accurate than monolingual recognition. The bilingual L2 advantage parallels word frequency effects in recognition and is attributed to the greater episodic distinctiveness of L2 words, relative to L1 words.