While most studies in the past focused on technical aspects of CP

While most studies in the past focused on technical aspects of CPR, recently the importance of non-technical factors such as teamwork, communication and leadership have been recognized [3-8]. Several studies have demonstrated that CPR causes significant mental stress in rescuers [9-15], and

health care workers often feel unprepared to manage stress and conflicts in a cardiac arrest situation [16-18]. This is important because the stress Inhibitors,research,lifescience,medical experienced in an emergency situation may impair performance. In line with that, we recently found that feeling stressed and overwhelmed while delivering CPR was associated with worse CPR performance [14]. Hence, stress reducing measures may improve performance in critical situations. To reduce stress, the focus on attentional processes may be a promising venue. Stress can have two opposite effects on attention. First, stress narrows attention Inhibitors,research,lifescience,medical [19].

For tasks that are relatively easy, narrowing attention can lead to improved performance by supporting a focus on the task [20]. However, narrowing of attention entails the danger of not noticing potentially Inhibitors,research,lifescience,medical important information, a phenomenon known as “tunnel vision” [21]. Furthermore, it is also related to premature closure, which is characterized by making decisions based on insufficient consideration of information available [22]. The second mechanism refers to an BAY 87-2243 chemical structure impaired ability to suppress irrelevant information, increased distractibility, ultimately leading to misjudgements Inhibitors,research,lifescience,medical of priorities [23-25]. A related mechanism refers to non-systematic scanning of informational cues [22]. However, information that

distracts from task priorities may not only stem from external events but may also be generated internally, for instance Inhibitors,research,lifescience,medical by worrying about one’s own performance (intrusive thoughts) [26]. Such intrusive thoughts may be increased by noticing that one’s performance is not optimal [27], thus possibly leading to a vicious circle. The response to acute stress is highly Adenylyl cyclase dependent on the individual’s perception of demands and resources [10,28], and on stress reactivity [29]. Therefore, stress management training may reduce stress [30]. Indeed, stress management training has had positive effects not only on stress indicators but also on performance [31,32]. However, although such procedures have been implemented in medical settings, they typically have not been evaluated in terms of medical performance [33]. Based on positive results achieved in other performance settings [15,32,34], and based on our recent finding that a brief leadership instruction improves CPR performance [5,35] we hypothesized that a brief task-focusing strategy may reduce stress and improve CPR performance.

These trials

demonstrated tumor specificity and adequate

These trials

demonstrated tumor specificity and adequate agent distribution with adverse effects similarly limited to target tissue damage and minimal to no systemic toxicity. These trials were limited, however, by the specificity of the delivered agents, which targeted only a subpopulation of tumor cells. Prior to our clinical trial, paclitaxel was the only conventional chemotherapeutic agent delivered via CED in a Inhibitors,research,lifescience,medical clinical trial [15]. This was mainly because paclitaxel does not cross the BBB, thus allowing the investigators to demonstrate that DW-MRI could be used to approximate the volume of distribution of CED. The trial resulted in a large incidence (40%) of chemical meningitis, a major drawback to the choice of paclitaxel [15, 16]. Though these studies highlighted initial challenges in the application of CED, they demonstrated the importance of careful and rational selection of agents for use in this method of delivery. 3. Early Experiences: CED of Integrase inhibitor Topotecan Our initial experience with

CED Inhibitors,research,lifescience,medical of antitumor agents utilized the cytotoxic agent topotecan. Topotecan is a camptothecin-class drug and acts as a topoisomerase-I inhibitor. It causes single-strand DNA breaks during DNA replication [17, 18]. This drug was selected after we demonstrated in vitro cytotoxicity Inhibitors,research,lifescience,medical against various malignant glioma cell lines [19]. Due to its activity in cells in the S-phase of division, topotecan is ideal for the treatment of mitotically active glioma cells

in the setting of relatively quiescent brain tissue. Previous experience with topotecan demonstrated poor penetration of the blood-brain barrier and significant dose-limiting toxicities, limiting systemic administration [20–23]. However, these same properties make it an ideal drug for administration Inhibitors,research,lifescience,medical via CED. In addition, an important aspect of the choice of topotecan was its effect on a vital Inhibitors,research,lifescience,medical cellular process, namely, the role of topoisomerase I on DNA processes. This focus on conventional chemotherapeutic agents as opposed to targeted therapies allows for greater coverage of heterogeneous glioma subpopulations. While targeted therapies can be successful in eliminating a specific subpopulation of PDK4 glioma cells that express a certain antigen, this provides a selective advantage for remaining neoplastic cells. Preclinical testing of topotecan that was performed in a model of glioblastoma was developed using a PDGF-B expressing retrovirus injected stereotactically into the adult white matter of rats to infect glial progenitors [19]. This resulted in the consistent development of tumors that closely resembled glioblastoma, with pseudopalisading necrosis, invasion, glomeruloid vascular proliferation, and survival of 14–19 days [24]. Topotecan was delivered using an implantable osmotic pump connected to an intracerebral infusion cannula (Alzet; Cupertino, CA) that was implanted into the tumor.

17,18 Fludeoxyglucose (FDG)-positron emission tomography (PET) sc

17,18 Fludeoxyglucose (FDG)-positron emission tomography (PET) scans, where blood flow and glucose utilization over different brain regions can be measured, may also provide useful information as to disease progression over time.19 Further, methods are improving to image amyloid plaques in

living patients using PET ligands that bind Aβ.20 These methods have been used to measure significant changes in amyloid deposition in patients with MCI.21 The most promising of these neuroimaging techniques and biochemical readouts could in time be used together as surrogate markers to provide an accurate assessment of disease state over time within Inhibitors,research,lifescience,medical an individual or across a population. There is a risk, however, of focusing too heavily on surrogate markers. In studies of rosiglitizone for diabetes, negative outcomes on disease appeared despite expected positive effects on the surrogate.22 Cholesterol has long been used as a surrogate for heart disease; however, in clinical Inhibitors,research,lifescience,medical trials of high-density lipoprotein-modifying drugs (such as torceptrabib) for prevention of heart disease, a positive effect on the surrogate was seen even though clinical outcomes were Inhibitors,research,lifescience,medical worsened.23 As in AD, these other chronic degenerative diseases have complex, multifactorial causes that are not necessarily reflected in the surrogate marker. Therefore, while using surrogate markers can be quite a meaningful

method to monitor aspects of disease progression, it is crucial to keep in mind the limitations of this approach. Understanding genetic risk factors for AD is another

method to facilitate early detection of high-risk individuals, while also providing insight into disease mechanisms. The discovery Inhibitors,research,lifescience,medical of genes underlying risk for AD has provided us with many of our most promising drug targets. Individuals with the apolipoprotein E4 allele (ApoE4), for example, have a significantly greater risk Inhibitors,research,lifescience,medical of developing Alzheimer’s disease, and often exhibit an earlier age of onset and a more aggressive form of the disease.24’1 While ApoE4 is a known risk factor for AD, we still do not fully understand its mechanism of function in AD pathogenesis. Identifying genetic subtypes of AD could allow for the buy G007-LK development of more individualized therapies, as well as aid in clinical trial design for novel drug therapies. In fact, in the Phase II trial for Bapineuzumab, a monoclonal Annual Review of Biochemistry antibody to β-amyloid developed by Wyeth and Elan, ApoE4 carriers were separated from noncarriers in the analysis. Only noncarriers demonstrated a significant benefit from the treatment, which would not have been detected had the population been analyzed as a whole.25 It is our hope that in the near future early detection techniques, such as measurements of Aβ load, neuroimaging analysis, and/or genetic testing will function much like cholesterol testing does for heart disease.

Both preclinical studies25 and clinical studies suggest that a mo

Both preclinical studies25 and clinical studies suggest that a more fine-grained multidimensional approach to impulsivity may be warranted and that nonplanning impulsivity may be a key ingredient of BPD. Aggression One of the more common impulsive behaviors evidenced by people with BPD are expressions of anger or reactive aggression. Thus, the kind of anger that is observed in BPD Inhibitors,research,lifescience,medical patients is an impulsive type of aggression, but the aggressive components may be analyzed by somewhat different measures than the impulsive components. For example, psychometric measures designed to measure aggression include the Buss-Perry Aggression Questionnaire (BPAQ),26 as well as measures

of life history of overt aggressive behaviors (life history of aggression [LHA]). Both measures have well-established psychometric properties and heritability has been established in twin studies using the Buss Durkee Hostility Interview (BDHI),27 a precursor of the BPAQ. Preliminary data also suggest that life history of aggression may be heritable. Laboratory Inhibitors,research,lifescience,medical paradigms that assess aggression behaviorally are available, including the Point Subtraction Aggression Paradigm (PSAP).28 In the PSAP, an experimental subject is instructed to accumulate “points” that can be exchanged for money and is told that they are playing in conjunction with a “confederate subject,” while in reality responses are generated by Inhibitors,research,lifescience,medical computer. Aggressive

responses are often retaliatory to provocations from the “confederate” and do not net the subject of the study HCS assay actual “points,” but may be initiated as an aggressive response to the perceived aggression of the confederate. Inhibitors,research,lifescience,medical The PSAP has been externally validated in violent and nonviolent male parolees and responses to this laboratory test have been correlated with other psychometric measures of aggression.29 The heritability of this laboratory measure has not been definitively established, but is being systematically assessed

in studies of twins Inhibitors,research,lifescience,medical (Coccaro et al, personal communication). Another laboratory test for evaluating the propensity for aggression in response to provocation is the Taylor Aggression Paradigm,30 in which aggressive responses to mild electric shocks are administered to the subject, ostensibly by a fictitious opponent. Aggressive behavior is evaluated as a function of the shock intensities administered by the subject Nature Reviews Cancer to this fictitious opponent. This paradigm has been used extensively in the evaluation of alcoholinduced aggression31 and has been applied to studies of reactive aggression in BPD (Coccaro et al, personal communication). Aggressive responding in the PSAP paradigm is a stable trait that can distinguish between aggressive and nonaggressive subjects, but, for both of these measures, the precise prevalence in specific personality disorders, such as BPD, and the degree of genetic influence on the PSAP has not been determined.

2003) While the

2003). While the olfactory systems and olfactory learning abilities of several species of slugs and Gamma-secretase activity snails have been extensively studied (Chase 1981, 1985; Chase and Tolloczko 1993; Gelperin 1994; Gervais et al. 1996; Sahley and Crow 1998; Balaban 2002), almost nothing is known about the anatomy and physiology of mucus trail chemosensation. This study identifies connections between Inhibitors,research,lifescience,medical the lip extensions that mediate mucus trail detection and the cerebral ganglia, and demonstrates that mucus stimuli detected by the lip extensions

are processed in the same central ganglia and in the same manner as odor molecules detected by the olfactory system. Our anatomical and tract-tracing experiments show that in the Euglandina, the nerve from the inferior tentacle joins with the nerve from the lip extension, and the combined nerve connects to the procerebral Inhibitors,research,lifescience,medical lobe where neurons from the lip extension synapse in the cell body layer. While a large swelling at the point where the lip extension nerve and oral tentacle nerve comes together suggests a ganglion, it is Inhibitors,research,lifescience,medical unlikely that afferent nerves from the sensory epithelium terminate at this point as nickel-lysine and Lucifer yellow taken up by the distal ends of lip extension nerve are transported past this point to the cerebral ganglion. Our results

suggest that the connectivity and processing of input from the lip extension may have arisen in the Euglandina as an elaboration of the neural processing dedicated to the oral tentacle in other snails and slugs. This hypothesis is supported by our observation Inhibitors,research,lifescience,medical that in Euglandina, the lip extension nerve and oral tentacle nerve join, and the joined nerve Inhibitors,research,lifescience,medical enters the cerebral ganglion in the mid-lateral area where the oral tentacle enters in other land snails. In the Euglandina, backfilling

the lip extension nerve produces extensive labeling of the procerebrum appearing to cover the entire procerebrum, and resembles the results of backfilling of the Cantareus olfactory nerve more than the Cantareus oral tentacle nerve backfilling. Labeling of the Euglandina olfactory nerve produces labeling of the procerebrum that looks substantially the same as the labeling produced by backfilling the lip extension nerve. In addition to the similarity Liothyronine Sodium between the anatomical labeling, the neuronal activity of the Euglandina procerebrum is similar to neuronal activity recorded from the procerebra of other land snails. The activity is characterized by a widespread oscillation in local field potential with a frequency of 0.1–0.3 Hz, and stimulation with odorants changes the frequency and amplitude of the oscillations (Chase 1981; Gelperin and Tank 1990; Kimura et al. 1993; Delaney et al. 1994; Ermentrout et al. 1998).

The clinical research sites were multidisciplinary outpatient cli

The clinical research sites were multidisciplinary outpatient clinics that offer brain health assessment and treatment services (such as EEG testing) for any medical condition. Expert clinicians at each site completed diagnostic interviews and were

blinded to the results of the BRISC and other self-report assessments. Recruitment This retrospective study recruited participants through advertising and self-referral. Inclusion criteria were in regard to the capacity to undergo a computerized test: reading at Year 5 level (equivalent to Year 6 in England and fifth grade in the United States), Inhibitors,research,lifescience,medical normal (or corrected to normal) vision, and ability to use a keyboard. The protocol received unlike independent ethics committee Inhibitors,research,lifescience,medical or institutional review board approval before recruitment of participants. All participants signed and dated an approved informed consent form. Where participants consented, these data have also been made available for open sharing and secondary analysis by the research community (Gordon et al. 2005, 2008). All research is in compliance with the Code of Ethics of the World Medical Association (Declaration of Helsinki). Main measures The assessment of behavioral health status At the testing site, participants Inhibitors,research,lifescience,medical first completed a computer battery of detailed

questions to provide an independent determination of behavioral health status. This assessment comprised established items to assess current or lifetime psychiatric and neurological conditions (Table 1). Stepwise stratification logic was used to determine “clinical” versus “healthy” behavioral health according to the criteria summarized in Figure Inhibitors,research,lifescience,medical 1. Figure 1 Summary of the criteria for independent classification of “good” versus “poor” brain health status. Table 1 Summary items Inhibitors,research,lifescience,medical used in the independent assessment of clinical versus healthy status The BRISC After the assessment of behavioral

health status, yet in the same testing session, participants completed the 45-question BRISC (Appendix 1) via computer, which took about 10 min to complete. The results provided one score for risk (negativity bias) and Batimastat two scores for coping (emotional resilience and social skills; Rowe et al. 2007; Williams et al. 2008). As indicated in Appendix 1, the 15-question mini version of the BRISC is made up of the five highest-loading BRISC items for each of the core content domains: negativity bias, emotional resilience, and social skills. Responses to each BRISC question were made on a scale of 1–5, with 5 representing higher functioning (less risk, better coping). We summed the responses for negativity bias, for emotional resilience, and for social skills (raw scores are shown in Appendix 2 for the 45-question BRISC and Appendix 3 for the mini-BRISC).

11 The development of AF in the absence of traditional risk facto

11 The development of AF in the absence of traditional risk factors, referred to as lone AF, suggested a potential role for genetics as a mediator of disease. Indeed, a family with lone AF transmitted with an autosomal dominant pattern of inheritance was first documented by Wolff in 1943.12 Epidemiological studies have found that individuals who have a first-degree AZD8055 solubility dmso relative with lone AF carry a 7- to 8-fold increased risk.13 Even more dramatic, the presence of an affected sibling was associated with a 70- and 34-fold increased risk in males and females, respectively.14 Inhibitors,research,lifescience,medical Although more pronounced in the context of

lone AF, the form of the arrhythmia associated with structural Inhibitors,research,lifescience,medical heart disease has also been shown to have a heritable component. A prospective cohort analysis from the Framingham Heart Study involving 2,243 subjects found that parental

AF conferred a 1.85-fold increased risk in offspring.15 A similar study from Iceland involving 5,269 patients corroborated the latter result, identifying a 1.77-fold increased risk of developing AF in first-degree relatives.16 This greater vulnerability is not attenuated by adjustment for traditional risk factors linked to the arrhythmia, suggesting that the heightened risk is secondary to an underlying genetic etiology.17 Collectively, these findings provide convincing epidemiological evidence Inhibitors,research,lifescience,medical to suggest that genetics play Inhibitors,research,lifescience,medical a critical role in the development of both

lone and structural AF. Mechanistic Subtype of AF 1: Gain-of-Function Potassium Channels and Enhanced Atrial Action Potential Repolarization The first causative gene responsible for familial AF was found in 2003. The culprit locus on this occasion was mapped to the short arm of chromosome 11 (11p15.5) in a four-generation Chinese family with an autosomal dominant pattern of inheritance for lone AF.18 Chromosome 11p15.5 was noted to contain the KCNQ1 gene, which encodes the poreforming α subunit of the slow component Inhibitors,research,lifescience,medical of the delayed rectifier potassium current (IKs). Loss-of-function mutations within KCNQ1 had previously been recognized as the cause for long QT syndrome type 1, a cardiac channelopathy associated with malignant ventricular arrhythmias and sudden cardiac death.19 Given its biological plausibility based on its established link with a cardiac arrhythmic http://www.selleckchem.com/products/yo-01027.html disorder, KCNQ1 was considered an ideal candidate gene. Sequencing of KCNQ1 identified a Ser140Gly mutation that segregated with AF cases within the family. Following identification of the putative culprit mutation, in vitro functional studies using COS-7 cells found that coexpression of mutant Ser140Gly KCNQ1 with KCNE1, the β subunit of IKs, resulted in markedly increased current density relative to the wild-type gene. These findings suggested that the Ser140Gly mutation resulted in a gain of function leading to increased IKs.

* The ML-1 binding site has high-affinity MEL receptors (K d<200

* The ML-1 binding site has high-affinity MEL receptors (K d<200 pM) with a consensus

rank order of drug potency in inhibiting [125I]MEL binding as follows: 2-iodomclatonin > 6-chloromelatonin ≥ MEL > 6-hydroxymelatonin > N-acetylserotonin >> 5-hydroxytryptamine. The ML-2 binding sites are characterized by a K d in the nanomolar range with a distinct pharmacological profile, notably a similar affinity for MEL and N-acetylserotonin.33,34 Due to difficulties in Inhibitors,research,lifescience,medical their characterization and to their low affinity, which does not seem to be compatible with the circulating MEL levels, less attention had been given to these ML-2 binding sites. The recent identification of MT, receptor reopens this question (see below). Molecular identification of MEL receptor subtypes The cloning of the first, high-affinity MEL receptor was a landmark in MEL receptor research history. This was achieved by using an expression cloning strategy to isolate the complementary Inhibitors,research,lifescience,medical DNA encoding for MEL receptor of Xenopus laevis dermal melanophores.35 This Xenoptis MEL receptor cDNA encoded a protein with seven putative transmembrane regions that led to its classification within the sellectchem superfamily of G-protein-coupled receptors.35 Identification of the Xenopus receptor sequence using homology-based screening methods led to the subsequent identification

of three types Inhibitors,research,lifescience,medical of vertebrate MEL Inhibitors,research,lifescience,medical receptors. Two receptor subtypes with highaffinity for MEL (initially termed Mel1a and Mel1b, but now called MT1 and MT2) have been cloned and characterized from sheep pars tuberalis (PT), human SCN and hamster and rat hypothalamus.36,37 In sheep PT, allelic isoforms of MT1 receptors (termed Mel1a(α) and Mel1a(β), respectively) have been identified.38 A third subtype of high-affinity

MEL receptor was cloned from a chicken brain library and termed the Mellc.39 The first Xenopus receptor to be isolated was a Inhibitors,research,lifescience,medical Mel1c receptor, which exists in two allelic isoforms, Mel1c(α) and Mel1c(β). So far, no mammalian homologue of the Mel1c receptor has been isolated, but cDNA fragments for a nonmammalian Mel1b have. All this molecular work has also demonstrated that, the characteristics of the high-affinity receptors are present in each of the three related receptors (MTl, MT2, and Mel1c). Very probably this is only the beginning GSK-3 of a long list. A receptor structurally related to the MEL receptors has already been isolated40,41 with a very interesting distribution of expression in neuroendocrine tissues.42 The natural ligand for this receptor has not. been identified (could it be a MEL metabolite?). More recently, a MEL receptor with a nanomolar affinity, called MT3, has also been isolated. The MT3 site is not a G-protein-coupled receptor, but corresponds to a binding on the enzyme quinone reductase.

31,32 Researchers have used related paradigms for producing gist

31,32 Researchers have used related paradigms for producing gist -based memory errors. For example, after studying patterns or shapes that are physically similar to a nonpresented prototype, participants later are likely to falsely recognize the novel prototype as a previously studied item.33,34 Similarly, after

studying numerous pictures or words Inhibitors,research,lifescience,medical from a particular category, people are likely to later show false recall or false recognition of nonpresented category members from the previously presented categories.35,36 While such responses are classified appropriately as memory distortions — people claim to remember items that they have never encountered before — those errors also reflect retention of useful information Inhibitors,research,lifescience,medical concerning the general themes, appearances, or meanings that participants did encounter. Retention of such information can facilitate the ability to generalize and abstract,9,16,17,37,38 and in that sense can be considered adaptive. Several kinds of experimental evidence support the idea that gist-based and associative memory errors indeed reflect the operation of adaptive processes. First, both associative and gist-based false recognition are reduced in patients with amnesic syndromes resulting from damage to the medial temporal lobes, thereby suggesting that such errors normally reflect the operation of a healthy Inhibitors,research,lifescience,medical Inhibitors,research,lifescience,medical memory

system.39-41 Second, recent studies have linked associative false recognition and creativity. In one study study, Howe et al42 presented DRM associate lists to children and adults before these participants attempted to solve compound remote associate task problems. Participants were presented with three word puzzles (eg, walk/beauty/over) and Inhibitors,research,lifescience,medical attempted to generate a solution word that is associated with all three target words (eg, sleep). When they were primed with DRM lists (eg, bed, rest, awake, tired, dream, etc) for

which the solution word on the buy BIBW2992 problem-solving task was the critical lure (eg, sleep), both children and adults showed improved performance on the problem-solving tasks compared with problems that were not primed by DRM lists. Importantly, however, this effect was observed only when participants falsely recalled the critical lure, thereby bolstering the authors’ claim that false memories can have beneficial effects on cognitive function under certain conditions. In another recent selleck products study linking creativity and associative false recognition, Dewhurst et al43 showed that susceptibility to DRM false recognition is predicted by performance on a remote associates task. This task is generally viewed as a measure of convergent thinking — a component of creativity that taps an individual’s ability to generate broad and numerous associations, and can thus be considered an adaptive cognitive process.

Key words: Glycogenosis II, GSDII, Pompe disease Glycogenosis II

Key words: Glycogenosis II, GSDII, Pompe disease Glycogenosis II (GSD II; Pompe’s disease; OMIM entry # 232300) is a storage disorder resulting from a deficiency of acid alpha-glucosidase, which is the only enzyme able to ZD1839 ic50 process glycogen into lysosomes. Enzyme deficiency leads to accumulation of glycogen in muscles, lysosomal disruption and excess of autophagic vesicle buildup inside

the myofibers, causing progressive cardiac, motor and respiratory failure (1). GSD II can be clinically divided into two main subtypes. The infantile form usually appears in the first month of life, presents with severe cardiac involvement Inhibitors,research,lifescience,medical and total deficiency of alpha-glucosidase activity (< 1% of normal controls), Inhibitors,research,lifescience,medical and progresses rapidly; the late-onset form is characterized by phenotypical variability even though the main findings are progressive muscle weakness and severe respiratory insufficiency (2, 3). Limb-girdle weakness is frequently the early sign of the late-onset disease. Patients usually report difficulty in walking and running, playing sports, climbing stairs or rising from a chair. Severe

weakness may also be observed in paraspinal muscles and additional neuromuscular features may include scapular winging and distal contractures. Inhibitors,research,lifescience,medical Respiratory muscles are always involved with weakness of the diaphragm, intercostal and Inhibitors,research,lifescience,medical accessory muscles whereas the cardiac damage is usually less severe (2). Muscle weakness and limited movement, especially of the antigravity muscles, may lead to alterations of posture, severe scoliosis and lumbar hyperlordosis, which entail biomechanical disadvantages, muscle contractures and deformity in a vicious circle of progressive disability. Increasing evidence shows that systemic abnormalities are present in GSDII Inhibitors,research,lifescience,medical patients and several tissues other than muscles may be involved in the course of the disease; therefore GSD II should be regarded as a multisystem disorder in which glycogen accumulation is present

in skeletal and smooth muscle, heart, liver, kidney, spleen, salivary glands, glial cells, brainstem nuclei, anterior horn cells of spinal cord and blood vessels (2). In this review we briefly summarize the non-skeletal muscle targets of the pathological selleck chemical process in late-onset GSD II. Nervous system involvement In Pompe mice, mass spectrometric quantification showed that glycogen progressively accumulates in brain (4). In pathological studies, glycogen storage was detected in cell bodies throughout the gray matter of the spinal cord, in cerebrum and cerebellum neurons, in glial and Schwann cells (5). Interestingly, animal models clearly showed glycogen accumulation in spinal and medullary respiratory neurons (6, 7). Glycogen storage was especially noted in phrenic motoneurons which also had larger soma area compared with wild-type controls (6).