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Neurofibromatosis, type I (NF-I) is associated with verbal and nonverbal neuropsychological deficits and neuroanatomical anomalies. Few relationships between CNS abnormalities and cognitive function in this population, however, have been found. Reading disabilities and developmental language impairments in the general population have been associated with particular morphologic features in inferior frontal gyrus (IFG) and Heschl's gyrus (HG). We compared the morphology of these regions in children with NF-I and controls. Verbal skills in NF-I were related to IFG morphology, such that individuals with NF-I who showed "typical" gyral patterns in the right hemisphere performed worse across language measures than those showing an extra "atypical" gyrus. A doubling of HG in the left and right hemispheres was also significantly associated with performance on several neuropsychological measures. This is the first study to link regional gyral morphology with language function in NF-I. A possible molecular basis for the observed relationships is discussed. [ABSTRACT FROM AUTHOR]

Pathogen-secreted polygalacturonases (PGs) alter plant cell wall structure by cleaving the α-(1 → 4) linkages between D-galacturonic acid residues in homogalacturonan (HG), macerating the cell wall, facilitating infection. Plant PG inhibiting proteins (PGIPs) disengage pathogen PGs, impairing infection. The soybean cyst nematode, Heterodera glycines, obligate root parasite produces secretions, generating a multinucleate nurse cell called a syncytium, a byproduct of the merged cytoplasm of 200-250 root cells, occurring through cell wall maceration. The common cytoplasmic pool, surrounded by an intact plasma membrane, provides a source from which H. glycines derives nourishment but without killing the parasitized cell during a susceptible reaction. The syncytium is also the site of a naturally-occurring defense response that happens in specific G. max genotypes. Transcriptomic analyses of RNA isolated from the syncytium undergoing the process of defense have identified that one of the 11 G. max PGIPs, GmPGIP11, is expressed during defense. Functional transgenic analyses show roots undergoing GmPGIP11 overexpression (OE) experience an increase in its relative transcript abundance (RTA) as compared to the ribosomal protein 21 (GmRPS21) control, leading to a decrease in H. glycines parasitism as compared to the overexpression control. The GmPGIP11 undergoing RNAi experiences a decrease in its RTA as compared to the GmRPS21 control with transgenic roots experiencing an increase in H. glycines parasitism as compared to the RNAi control. Pathogen associated molecular pattern (PAMP) triggered immunity (PTI) and effector triggered immunity (ETI) components are shown to influence GmPGIP11 expression while numerous agricultural crops are shown to have homologs., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Masson SAS.. All rights reserved.)

The plant cell wall structure can be altered by pathogen-secreted polygalacturonases (PGs) that cleave the α-(1→4) linkages occurring between D-galacturonic acid residues in homogalacturonan. The activity of the PGs leads to cell wall maceration, facilitating infection. Plant PG inhibiting proteins (PGIPs) impede pathogen PGs, impairing infection and leading to the ability of the plant to resist infection. Analyses show the Glycine max PGIP11 ( GmPGIP11 ) is expressed within a root cell that is parasitized by the pathogenic nematode Heterodera glycines , the soybean cyst nematode (SCN), but while undergoing a defence response that leads to its demise. Transgenic experiments show GmPGIP11 overexpression leads to a successful defence response, while the overexpression of a related G. max PGIP, GmPGIP1 does not, indicating a level of specificity. The analyses presented here have identified PGIPs from 51 additional studied proteomes, many of agricultural importance. The analyses include the computational identification of signal peptides and their cleavage sites, O -, and N -glycosylation. Artificial intelligence analyses determine the location where the processed protein localize. The identified PGIPs are presented as a tool base from which functional transgenics can be performed to determine whether they may have a role in plant-pathogen interactions., (Published by Elsevier Inc.)

Background: Miscanthus, a C4 member of Poaceae, is a promising perennial crop for bioenergy, renewable bioproducts, and carbon sequestration. Species of interest include nothospecies M. x giganteus and its parental species M. sacchariflorus and M. sinensis. Use of biotechnology-based procedures to genetically improve Miscanthus, to date, have only included plant transformation procedures for introduction of exogenous genes into the host genome at random, non-targeted sites., Results: We developed gene editing procedures for Miscanthus using CRISPR/Cas9 that enabled the mutation of a specific (targeted) endogenous gene to knock out its function. Classified as paleo-allopolyploids (duplicated ancient Sorghum-like DNA plus chromosome fusion event), design of guide RNAs (gRNAs) for Miscanthus needed to target both homeologs and their alleles to account for functional redundancy. Prior research in Zea mays demonstrated that editing the lemon white1 (lw1) gene, involved in chlorophyll and carotenoid biosynthesis, via CRISPR/Cas9 yielded pale green/yellow, striped or white leaf phenotypes making lw1 a promising target for visual confirmation of editing in other species. Using sequence information from both Miscanthus and sorghum, orthologs of maize lw1 were identified; a multi-step screening approach was used to select three gRNAs that could target homeologs of lw1. Embryogenic calli of M. sacchariflorus, M. sinensis and M. x giganteus were transformed via particle bombardment (biolistics) or Agrobacterium tumefaciens introducing the Cas9 gene and three gRNAs to edit lw1. Leaves on edited Miscanthus plants displayed the same phenotypes noted in maize. Sanger sequencing confirmed editing; deletions in lw1 ranged from 1 to 26 bp in length, and one deletion (433 bp) encompassed two target sites. Confocal microscopy verified lack of autofluorescence (chlorophyll) in edited leaves/sectors., Conclusions: We developed procedures for gene editing via CRISPR/Cas9 in Miscanthus and, to the best of our knowledge, are the first to do so. This included five genotypes representing three Miscanthus species. Designed gRNAs targeted all copies of lw1 (homeologous copies and their alleles); results also confirmed lw1 made a good editing target in species other than Z. mays. The ability to target specific loci to enable endogenous gene editing presents a new avenue for genetic improvement of this important biomass crop., (© 2022. The Author(s).)

Intervention-related changes in spatiotemporal profiles of regional brain activation were examined by whole-head magnetoencephalography in 15 children with severe reading difficulties who had failed to show adequate progress to quality reading instruction during Grade 1. Intensive intervention initially focused on phonological decoding skills (for 8 weeks) and, during the subsequent 8 weeks, on rapid word recognition ability. Clinically significant improvement in reading skills was noted in 8 children who showed "normalizing" changes in their spatiotemporal profiles of regional brain activity (increased duration of activity in the left temporoparietal region and a shift in the relative timing of activity in temporoparietal and inferior frontal regions). Seven children who demonstrated "compensatory" changes in brain activity (increased duration of activity in the right temporoparietal region and frontal areas, bilaterally) did not show adequate response to intervention. Nonimpaired readers did not show systematic changes in brain activity across visits.

This longitudinal study examined the development of the brain mechanism involved in phonological decoding in beginning readers using magnetic source imaging. Kindergarten students were assigned to 2 groups: those who showed mastery of skills that are important predictors of proficient reading (low-risk group) and those who initially did not show mastery but later benefited from systematic reading instruction and developed average-range reading skills at the end of Grade 1 (high-risk responders). Spatiotemporal profiles of brain activity were obtained during performance of letter-sound and pseudoword naming tasks before and after Grade 1 instruction. With few exceptions, low-risk children showed early development of brain activation profiles that are typical of older skilled readers. Provided that temporoparietal and visual association areas were recruited into the brain mechanism that supported reading, the majority of high-risk responder children benefited from systematic reading instruction and developed adequate reading abilities., (Copyright (c) 2005 APA, all rights reserved.)

Hemodynamic brain imaging and lesion studies have suggested differential involvement of expressive language-related cortical regions based on the phonemic versus semantic characteristics of verbal cues. The aims of this study were: 1) to elucidate the relative timing of the activity of inferior frontal and anterior insular versus motor and supplementary motor cortex during a fluency task and 2) to assess potential differences in the location or timing of activity in anterior and posterior language areas based on letter versus category cues. Using magnetic source imaging (MSI), we found significantly earlier onset latencies and a greater number of activity sources in motor and supplementary motor compared with inferior frontal and anterior insular regions. We also observed greater left versus right hemispheric asymmetry of activation for letter compared with category cues. This study provides new insights into cortico-cortical interactions during expressive language tasks.

We used magnetic source imaging (MSI) to investigate the spatio-temporal patterns of brain activity associated with line bisection judgments and double simultaneous visual stimulation in 14 healthy adults. Consistent with lesion and hemodynamic neuroimaging studies, we found the greatest number of activity sources in right inferior parietal cortex. These sources were most prominent, on average, between 200 and 300 ms after the onset of single (left, right, or center) target stimuli. A greater number of significant activity sources were found in right inferior parietal, occipital, and prefrontal cortices during bilateral compared with unilateral stimulus presentation. Based on these observations, we suggest that a more parsimonious physiological explanation of visual extinction than the hemispheric rivalry account may be the additional neuronal excitation required in right occipital and parietal cortices for accurate bilateral visual perception.

Object: In this study the authors evaluated the sensitivity and selectivity of a noninvasive language mapping procedure based on magnetoencephalography (MEG), for determining hemispheric dominance for language functions., Methods: Magnetic activation profiles of the brain were obtained from 100 surgical candidates (age range 8-56 years) with medically intractable seizure disorder by using a whole-head MEG system within the context of a word recognition task. The degree of language-specific activity was indexed according to the number of consecutive sources (modeled as single, moving current dipoles) in perisylvian brain areas. Only activity sources that were observed with a high degree of spatial and temporal overlap in two split-half data sets were used to compute the MEG laterality index. Independently, all patients underwent Wada testing for the determination of hemispheric dominance for language. Independent clinical judgments based on MEG and Wada data showed a high degree of concordance (87%). Magnetoencephalography laterality judgments had an overall sensitivity of 98%, but a lower selectivity of 83%, which was due to the fact that MEG detected more activity in the nondominant hemisphere than was predicted based on the Wada test. A number of objective criteria were derived based on this large patient series to ensure data quality and bolster the clinical usefulness of MEG for language mapping., Conclusions: Although the availability of MEG is still limited across epilepsy surgery centers, this study method may be substituted for the Wada procedure in assessing hemispheric dominance for language in select cases.

Objective: The authors evaluated the sensitivity and selectivity of interictal magnetoencephalography (MEG) versus prolonged ictal and interictal scalp video-electroencephalography (V-EEG) in order to identify patient groups that would benefit from preoperative MEG testing., Methods: The authors evaluated 113 consecutive patients with medically refractory epilepsy who underwent surgery. The epileptogenic region predicted by interictal and ictal V-EEG and MEG was defined in relation to the resected area as perfectly overlapping with the resected area, partially overlapping, or nonoverlapping., Results: The sensitivity of a 30-minute interictal MEG study for detecting clinically significant epileptiform activity was 79.2%. Using MEG, we were able to localize the resected region in a greater proportion of patients (72.3%) than with noninvasive V-EEG (40%). MEG contributed to the localization of the resected region in 58.8% of the patients with a nonlocalizing V-EEG study and 72.8% of the patients for whom V-EEG only partially identified the resected zone. Overall, MEG and V-EEG results were equivalent in 32.3% of the cases, and additional localization information was obtained using MEG in 40% of the patients., Conclusion: MEG is most useful for presurgical planning in patients who have either partially or nonlocalizing V-EEG results.

Considerable evidence supports the idea of magnetoencephalography (MEG) being a valuable noninvasive tool for presurgical mapping of sensory and motor functions. In this study, we test the validity and replicability of a new experimental paradigm for simultaneous sensory and motor mapping using MEG recordings. This comprehensive sensorimotor protocol (CSSMP), where external mechanic stimulation serves as a cue for voluntary movements, allows the recording of sensory and motor cortical responses during a single activation task. The stability and replicability of MEG-derived recordings during this paradigm were tested in a group of eight neurologically normal volunteers and six patients with perirolandic lesions. We found that a common sensorimotor cortical network, engaging sensory (S1, S2) and motor (M1) areas, was reliably activated in all subjects and patients and that the results remained exceptionally stable over time. Additionally, the clinical validity of the MEG-derived maps of activation was tested through intraoperative electrocortical stimulation mapping in the group of patients. The MEG-derived anatomical maps for specific sensory (S1) and motor (M1) responses were verified, by direct cortical mapping, and confirmed with the patient's surgical outcome. The results of this validation study show that the so-called CSSMP is a reliable and reproducible method for assessing simultaneously sensory and motor areas. This method minimizes methodological problems and improves our knowledge of the spatiotemporal organization of the sensorimotor cortical network and helps to optimize the surgical management of patients with perirolandic lesions.

Visual-spatial impairment and neuroanatomical abnormalities are considered hallmark features of neurofibromatosis, type I (NF-I). Numerous studies have demonstrated visual-spatial deficits in children with NF-I, but few relations between these deficits and neuroanatomical abnormalities have been identified. We compared the functional neuroanatomy of cerebral regions involved in the spatial transformation of alphanumeric stimuli in individuals with NF-I and healthy control participants using functional magnetic resonance imaging (fMRI). Given the prevalence of visual pathway abnormalities and visual-spatial deficits in NF-I, we hypothesized that less neuronal hemodynamic activity would be found in occipital and parietal cortices in this group compared with controls. However, NF-I participants relied to a greater degree than controls on posterior cortex (including occipital, parietal, and middle temporal cortices) relative to lateral and inferior frontal regions during visual-spatial analysis. This pattern was significantly related to their behavioral performance on the fMRI task, which in turn was also positively correlated with reading scores. These findings support evidence of frontal cortical anomalies in NF-I and may provide a pathophysiological basis for cognitive deficits in NF-I.

Neurofibromatosis 1 is associated with reading disabilities, but few associations between neuroanatomic abnormalities and reading problems have been found. We examined the neuronal bases for phonologic processing, a core component of learning to read, in 15 individuals with neurofibromatosis 1 and 15 controls using functional magnetic resonance imaging (MRI). Our results revealed differential use of inferior and dorsolateral prefrontal cortical areas relative to posterior (temporal, parietal, and occipital) cortices for participants with neurofibromatosis 1 compared with controls during phonologic (rhyme) decisions. In addition, similar to previous brain imaging studies of reading deficits in the general population, poorer performance on one of the phonologic decision tasks was associated with increased signal change in the right superior temporal gyrus for the neurofibromatosis 1 group. Behavioral performance on the functional MRI tasks was related to academic reading measures for the neurofibromatosis 1 group. The differential patterns of functional connectivity observed here lend support to previous morphologic studies that suggested inferior frontal and superior temporal areas to be important mediators of reading and language development in neurofibromatosis 1.

Neurofibromatosis type-1 (NF1) is a common genetic disorder associated with a variety of medical complications, cognitive impairments, and behavioral problems including a high incidence of Attention Deficit Hyperactivity Disorder (ADHD). The current study examined the hypotheses that deficits in visual-spatial/motor abilities enable one to discriminate and classify children with NF1 (n = 101) compared to control children (n = 37), beyond effects secondary to parent reported ADHD symptomology. Discriminant analysis showed a multivariate combination of visual-spatial/motor ability tests (Judgment of Line Orientation, Block Design subtest of the WISC-III, Recognition-Discrimination Test, Beery Visual-Motor Integration Test) to be a significant predictor of NF1 diagnostic status (P = 0.0000004; canonical R(2) = 0.2306). A significantly greater degree of ADHD behavior was found in the NF1 group, and a discriminant analysis using ADHD residualized visual-spatial motor scores indicated that the combination of tests continued to be a significant predictor of group membership after the level of ADHD behavior was controlled (P = 0.00002 and a canonical R(2) = 0.1818). This combination of tests proved to be a strong discriminator of NF1. It correctly identified 90% of individuals with the diagnosis, and may be useful to educators to provide assistance and alternatives to minimize the impact of learning problems in those with either known or suspected NF1., (Copyright 2003 Wiley-Liss, Inc.)

Clinical diagnosis of attention-deficit hyperactivity disorder (ADHD) is based on evaluation of behavioral functioning in three domains: inattentiveness, hyperactivity, and impulsivity. Caudate and frontal lobe function figures prominently in several neuroanatomic models of attentional functioning. Studies comparing children with and without ADHD have found differences in the size and symmetry of the caudate nuclei. Using multiple regression, we tested the hypothesis that caudate volume symmetry (log left minus log right caudate volume) measured from serial sagittal magnetic resonance images in a sample of nonreferred children (12 girls/15 boys, 7.0 to 16.6 years, 81 to 129 IQ) would predict the cumulative severity of parent-reported ADHD diagnostic behaviors beyond variance predicted by age, sex, and level of internalizing problems as measured by the Child Behavior Checklist. No child had been previously diagnosed with ADHD, although one child was found to meet diagnostic criteria based on the rating scale used for the study. The degree of caudate asymmetry significantly predicted cumulative severity ratings of inattentive behaviors (P = .015), uniquely accounting for 17.1% of the variance in inattention symptomatology over demographic variables and internalizing problems, which collectively predicted 28.9% of the variance. Caudate asymmetry uniquely accounted for only 4.3% of the variance in cumulative severity ratings of hyperactive/impulsive symptomatology over demographic variables and internalizing problems that collectively predicted 21.2% of the variance. A greater degree of right to left caudate volume asymmetry predicted subclinical inattentive behaviors in a sample of nonreferred children. This finding is congruent with neuroanatomic models of attention emphasizing lateralized alteration in prefrontal/striatal systems. The results support the view that clinical ADHD is the extreme of a behavioral continuum that extends into the normal population.

Developmental trajectories of two classes of human memory, implicit and explicit memory, appear to diverge. We examined how developmental differences in perceptual and conceptual priming, two types of implicit memory, coincide with differences between familiarity and recollective responses on explicit memory tests that employ the Remember/Know paradigm ( Tulving, 1985 ). Both types of priming were characterized by developmental invariance in 52 children and adolescents ages 8-19 years. Contrary to Komatsu, Naito, and Fuke (1996) results, few age-group differences in perceptual priming were observed following a levels-of-processing encoding manipulation. In contrast, age group differences were found for "Remember" but not "Know" responses. Neither levels of awareness nor strategies influenced priming. Adult levels of performance appear earlier in development on perceptual and conceptual priming tests compared to explicit memory tests. Similar developmental dissociations exist between explicit and implicit memory performance as between "Remember" and "Know" recollective responses.

Background: Neurofibromatosis type 1 (NF-1) is associated with learning disabilities and cognitive impairment in childhood and adolescence. Individuals with NF-1 have a propensity for brain hyperintensities on T2-weighted magnetic resonance images, macrocephaly, and optic gliomas. Few clear relationships between these central nervous system abnormalities and cognitive function, however, have been found in this population., Objectives: To determine whether planum temporale (PT) and planum parietale (PP) morphologic features are associated with learning disabilities in NF-1., Patients and Methods: We measured and compared the surface area, gray matter volume, and asymmetry of the PT and PP on T1-weighted MRIs from 24 children and adolescents with NF-1 and an equal number of controls. Relationships between these measurements and cognitive and academic achievement scores were examined., Results: The left PT in boys with NF-1 was significantly smaller in both surface area and gray matter volume compared with girls with NF-1 and controls. Boys with NF-1 also showed greater symmetry between the left and right hemispheres in this region compared with girls with NF-1 and controls, who showed a pattern of left greater than right asymmetry of the PT. Intelligence-based discrepancy scores of reading and math achievement, which are commonly used to define learning disabilities, were significantly related to PT asymmetry in the NF-1 group as a whole. Less leftward asymmetry of the PT was associated with poorer reading and math achievement in relation to intellectual test scores., Conclusions: The high susceptibility of individuals with NF-1 to develop reading and other learning disabilities seems to be related to the development of the sylvian fissure. These results provide further support for the hypothesized association between sylvian fissure morphologic features and learning disabilities.

Although clinical syndromes of visual-spatial neglect have been well described in adults, clinical features of neglect associated with subcortical dysfunction are infrequently reported in children and have not been described in detail. Unilateral visual-spatial neglect in a 7-year-old male following removal of a right subcortical juvenile pilocytic astrocytoma is reported. Preoperative baseline neurocognitive assessment of the patient established intact attentional and intellectual functioning. Postoperatively visual-spatial neglect was observed that was not accounted for by the patient's visual field deficit. Consistent with classic features of attentional neglect, increases in attentional demands led to greater errors in performance. The risk of unilateral neglect following resection of subcortical tumors that abut the thalamus or disrupt thalamo-cortical projections was confirmed in a retrospective analysis of patients referred for neurocognitive testing at our site. It was concluded that the ventral thalamus may play a role in visual-spatial attention early in development.

Explicit memory appears to be supported by medical temporal lobe structures, whereas separate neocortical regions may mediate perceptual and conceptual implicit memory. Children and adults with temporal lobe epilepsy (TLE) and matched controls were administered experimental verbal memory tests. Performance on implicit tests--word identification and word generation--was contrasted with explicit recognition and recall. Encoding conditions emphasized either conceptual or perceptual aspects of study words and were crossed with presentation modality. The priming performance of participants with TLE did not differ from controls, but participants with TLE did show deficits on recognition and recall measures. Thus, intact left temporal cortex does not appear to be necessary for normal implicit memory performance, even when conceptual processing is emphasized at study or test.

Phonological and semantic aspects of language were examined in patients with unilateral temporal lobe epilepsy (TLE) and healthy controls using functional MRI. We expected to replicate previous findings in healthy individuals showing relatively greater activation in frontal regions for phonological compared with semantic processing, and greater activation in temporal regions for semantic compared with phonological processing. We hypothesized that differences between patients with left TLE and healthy controls would be found in the pattern of left temporal cortical activation associated specifically with semantic processing. Patients with right TLE were included as a seizure control group. All TLE patients previously showed left hemisphere language dominance on intracarotid sodium amytal studies. Greater blood oxygen level dependent activation was found during phonological processing compared with semantic processing in frontal regions for healthy participants but, contrary to expectation, semantic processing did not lead to increased temporal lobe activity relative to phonological processing. Furthermore, no differences between left temporal patients and controls were found specifically in left temporal cortex. Rather, patients with left temporal seizure foci showed significantly greater left dorsolateral prefrontal activity compared with controls, as well as increased signal change in left inferior frontal and right middle temporal gyrus. Surprisingly, patients with right, but not left, TLE showed poorer performance on the linguistic tasks compared with controls, as well as a decrease in right superior temporal activation. The results converge with studies of dyslexic patients showing increased left frontal activity in the presence of left temporal dysfunction and are suggestive of both inter- and intra-hemispheric functional reorganization of language representation in left TLE.

Attentional control in children and adolescents with unilateral temporal-lobe excisions was examined using two experimental tasks, a lexical-decision and a spatial-cue task, and a standardized vigilance task. Participants with left temporal excisions took longer than controls to reorient their attention after invalid cues compared to neutral cues in the lexical task and they made more errors on all three of the tasks. Participants with right temporal excisions differed from controls in the number of errors made on the spatial task. No differences were found between the lesion and control groups on reaction-time measures of the spatial task. The results suggest that a material-specific inhibition impairment, as well as a more general sustained attention deficit, may result after a left temporal excision in childhood or adolescence. These deficits are considered in the context of neuroanatomical models of attentional control.