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Novel approaches and applications in identifying DNA methylation markers of cardio-kidney-metabolic disease.

Cardio-kidney-metabolic (CKM) diseases represent a major public health challenge, accounting for a large proportion of global burden of morbidity and mortality. These conditions share risk factors, including genetic predisposition, environmental exposures, and lifestyle influences, which collectively drive disease development and progression. Epigenetic modifications, particularly DNA methylation (DNAm), serve as key mediators and biomarkers between these risk factors and disease phenotypes by regulating gene expression without altering the DNA sequence. Epigenome-wide association studies have identified DNAm markers associated with CKM diseases and related phenotypes, highlighting both shared pathways and disease-specific epigenetic signatures in inflammation, metabolic dysfunction, and aging-related processes. Longitudinal studies further demonstrate the dynamic nature of DNAm changes over time, offering insights into disease trajectories. Additionally, methylation risk scores integrating multiple epigenetic markers show promise in improving disease prediction and risk stratification beyond traditional clinical factors. To synthesize the current evidence, we conducted a targeted literature search in PubMed for English-language, peer-reviewed articles published between 2014 and the present. Future research leveraging large, well-phenotyped cohorts, advanced statistical methods, and innovative study designs will be critical for uncovering novel biomarkers, refining risk prediction models, and developing targeted epigenetic therapies to mitigate the global burden.

Humans↗

The effects of impaired trace element status on polymorphonuclear leukocyte activation in the development of vascular complications in type 2 diabetes mellitus.

Impaired trace element metabolism may be involved in some of the metabolic dysfunctions, and contribute to the development of vascular complications in diabetic patients. In order to investigate the relationships among diabetes mellitus, trace element status, leukocyte activation and vascular complications, 55 type 2 diabetic patients (34 with vascular complications and 21 without vascular complications) and 50 non-diabetic control subjects were studied. The mean leukocyte count (p<0.001), polymorphonuclear elastase (p<0.001), erythrocyte malondialdehyde (p<0.001), and glycated haemoglobin (p<0.001) levels, and copper/ zinc ratio (p<0.001) were found to be higher in diabetic patients than in the control group, but serum zinc levels (p<0.001) and erythrocyte superoxide dismutase activities (p<0.001) were lower, and serum copper levels showed no differences. In patients with vascular complications, the mean leukocyte count (p<0.05), zinc (p<0.05), polymorphonuclear elastase (p<0.05), erythrocyte malondialdehyde (p<0.001) and glycated haemoglobin (p<0.05) levels, and copper/zinc ratio (p<0.001) were significantly different from those patients without complications. Closer correlations between the copper/zinc ratio and polymorphonuclear elastase (r=0.82, p<0.01), erythrocyte malondialdehyde (r=0.46, p<0.05) or erythrocyte superoxide dismutase (r= -0.85, p<0.01) were found in patients with vascular complications compared to those without, and all of those showed significant relationships with poor glycaemic metabolic control. We conclude that zinc deficiency may provoke polymorphonuclear leukocyte activation, and contributes to the development of vascular complications in type 2 diabetic patients. Furthermore, copper/zinc ratio and polymorphonuclear elastase may be used as important markers to evaluate the presence of vascular complications.

Adult↗

The effect of copper supplementation on the concentration of copper in the brain of the brindled mouse.

The brindled mutant mouse is a useful model to study Menkes kinky-hair syndrome. The metabolic dysfunctions in both human and rodent are related to insufficient levels of bioavailable copper. Recently, copper supplementation therapy has been able both to prevent the appearance of various neuropathological changes and to prolong the life of these mutant mice. The optimum conditions for supplementation have been shown to be two intraperitoneal injections on postnatal days 7 and 10. The present study reports on the brain copper concentrations before, during and after the intraperitoneal copper therapy. The results demonstrate that postnatal days 7 and 10 correspond to two important epochs in copper homoeostasis. The supplementation therapy seems to provide sufficient bioavailable copper to respond to the needs of the animal at these crucial time points. The results are discussed in terms of their importance to the human copper disorder.

Age Factors↗

Role of nitric oxide in regulation of coronary blood flow during myocardial ischemia in dogs.

OBJECTIVES: This study was undertaken to examine whether nitric oxide released in ischemic myocardium decreases the coronary vascular resistance and attenuates the severity of contractile and metabolic dysfunction. BACKGROUND: Endothelium-derived relaxing factor, recently identified as nitric oxide, is a potent relaxant of coronary smooth muscle. METHODS: The left anterior descending coronary artery was perfused through an extracorporeal bypass tube placed in the carotid artery in 56 open chest dogs. After hemodynamic stabilization, we occluded this bypass tube to decrease coronary blood flow to one third of the control flow. Thereafter, we maintained a constant coronary perfusion pressure (40.9 +/- 3.1 mm Hg). RESULTS: Under ischemic conditions, the coronary arteriovenous differences in nitrate and nitrite (end products of nitric oxide) increased (from 3.5 +/- 0.4 [mean +/- SEM] to 12.9 +/- 2.1 mumol/liter, p < 0.01). NG-Monomethyl L-arginine (3 micrograms/kg body weight per min, intracoronary) decreased the coronary arteriovenous differences in nitrate and nitrite (5.0 +/- 0.9 mumol/liter, p < 0.05) and coronary blood flow (from 29.8 +/- 0.5 to 18.1 +/- 1.1 ml/100 g per min, p < 0.001). Fractional shortening (from 3.7 +/- 1.0 to -1.3 +/- 0.7%, p < 0.001) and lactate extraction ratio (from -44.0 +/- 4.1 to -59.2 +/- 4.9%, p < 0.005) of the perfused area also decreased. These values were restored by the concomitant administration of L-arginine. Blood flow to the endomyocardium was decreased relative to the epimyocardium. A reduction in coronary blood flow and worsening of myocardial contractile and metabolic functions due to the administration of NG-monomethyl L-arginine during ischemia were observed in denervated hearts. A reduction in coronary blood flow in ischemic myocardium was observed with the administration of NW-nitro-L-arginine methyl ester as well, although neither NW-nitro-L-arginine methyl ester nor NG-monomethyl L-arginine changed coronary blood flow and myocardial contractile and metabolic functions in the nonischemic myocardium. The cyclic guanosine monophosphate content of epicardial coronary artery increased due to myocardial ischemia; this increase was attenuated with NG-monomethyl L-arginine treatment. CONCLUSIONS: We conclude that endogenous nitric oxide predominantly decreases the coronary vascular resistance of ischemic endomyocardium, thereby improving myocardial contractility and metabolic function.

Animals↗

The effects of thyroid hormone level and action in developing brain: are these targets for the actions of polychlorinated biphenyls and dioxins?

Alterations in thyroid hormone level or responsivity to thyroid hormone have significant neurologic sequelae throughout the life cycle. During fetal and early neonatal periods, disorders of thyroid hormone may lead to the development of motor and cognitive disorders. During childhood and adult life, thyroid hormone is required for neuronal maintenance as well as normal metabolic function. Those with an underlying disorder of thyroid hormone homeostasis or mitochondrial function may be at greater risk for developing cognitive, motor, or metabolic dysfunction upon exposure to substances which alter thyroid hormone economy. Polychlorinated biphenyls (PCBs) and dioxins have been argued to interfere with thyroid hormone action and thus may affect the developing and mature brain. Animal models provide useful tools for studying the effects of thyroid hormone disorders and the effects of environmental endocrine disruptors. The congenitally hypothyroid, hyt/hyt, mouse exhibits abnormalities in both the cognitive and motor systems. In this mouse and other animal models of thyroid hormone disorders, delayed somatic and reflexive development are noted, as are permanent deficits in hearing and locomotor and adaptive motor behavior. This animal's behavioral abnormalities are predicated on anatomic abnormalities in the nervous system. In turn, these abnormalities are correlated with differences in neuronal structural proteins. In normal mice, the expression of mRNAs coding for these proteins occurs temporally with the onset of autonomous thyroid hormone production. The hyt/hyt mouse has a mutation in the thyroid stimulating hormone receptor (TSHr) gene which renders it incapable of transducing the TSH signal in the thyrocyte to produce thyroid hormone. Some behavioral and possibly some biochemical abnormalities in mice exposed to PCBs are similar to those seen in the hyt/hyt mouse. In addition to direct effects on brain development and neuronal maintenance, thyroid hormone is necessary for maintaining metabolic functioning through its influence on mitochondria. Because the brain is particularly sensitive to inadequate energy generation, disorders of thyroid hormone economy also indirectly impair brain functioning. Alterations in thyroid hormone level result in differing expression of mitochondrial genes. Mutations in these mitochondrial genes lead to well-recognized syndromes of encephalomyopathy, myopathy, and multisystem disorder. Hence, PCBs and dioxins, by possibly altering the thyroid hormone milieu, may alter the functioning of mitochondria in the generation of adenosine triphosphate (ATP). The use of animal models of thyroid hormone deficiency for behavioral, anatomic, histologic, and molecular comparison will help elucidate the mechanisms of action of these putative endocrine-disrupting compounds. The study of thyroid hormone disorders provides a template for relating thyroid hormone mediated effects on the brain to these compounds.

Animals↗

Hypothermia, hypoglycemia, and hypothyrosis associated with poult enteritis and mortality syndrome.

A metabolic dysfunction contributes to the poor performance and mortality associated with Poult Enteritis and Mortality Syndrome (PEMS). Within 2 d after contact-exposed poults were removed from the presence of PEMS-infected poults and returned to their respective treatment rooms to infect experimental poults, the experimental poults began to huddle together and show signs of the disease. When separated from the huddle, body temperatures of exposure poults were depressed significantly. Body temperatures decreased progressively through 8 d after exposure with a maximum depression of 2 C and returned to a normal level at 18 d after PEMS exposure. Similar decreasing patterns in serum glucose, inorganic phosphorus, triiodothyronine, and thyroxine were observed, with maximum decreases in these serum constituents being found between 8 and 13 d after PEMS exposure. There were significant correlations among decreasing body temperatures, decreasing serum constituents, and mortality in the PEMS-exposed poults. Daily mortality rates associated with PEMS began at 6 d and peaked at 9 d after PEMS exposure. Mortality rates decreased from 9 to 15 d after experimental PEMS exposure. Depressions in serum constituents, body temperature, and increased mortality rates did not coincide with decreased feed intake associated with PEMS. Therefore, it was concluded that the agent(s) causing PEMS may have a direct effect on energy metabolism in afflicted poults.

Animals↗

Inhibition of glyceraldehyde-3-phosphate dehydrogenase in post-ischaemic myocardium.

OBJECTIVE: Myocardial reperfusion following brief period of ischaemic is associated with prolonged, reversible periods of metabolic dysfunction. As the glycolytic enzyme glyceraldehyde-3-phosphate dehydrogenase (GAPDH) is inhibited in vitro by reactive oxygen species, we hypothesized that production of reactive oxygen species during reperfusion would lead to inhibition of GAPDH in post-ischaemic myocardium. METHODS: Anaesthetized closed-chest-dogs were subjected to 20 min balloon occlusion of the left anterior descending coronary artery. Biopsy samples were taken after 3 and 24 h of reperfusion, to determine the activity of GAPDH and the concentrations of glycolytic intermediates in post-ischaemic and remote, non-ischaemic territories. RESULTS: A significant reduction in GAPDH activity was observed in post-ischaemic relative to remote tissue after 3 h reperfusion (4.8 +/- 0.5 vs. 2.9 +/- 0.2 mumol/min/mg protein; P < 0.01). Western blotting revealed no reduction in the levels of GAPDH protein. Analysis of enzyme kinetics showed the loss of activity to be associated with decreased Vmax (5.9 +/- 0.5 vs. 3.2 +/- 0.2 mumol/min/mg protein; P < 0.01) with no significant change in the Km for glyceraldehyde-3-phosphate (GAP). Incubation of the inhibited enzyme under both mild and strong reducing conditions failed to reactivate the enzyme. The acute reduction in enzyme activity in post-ischaemic tissue was accompanied by regional differences in glycolytic intermediates, notably a twofold accumulation of GAP (P < 0.05), and a reduction in the glucose metabolic rate (GMR) determined by positron emission tomography and [18F]2-fluorodeoxyglucose. By 24 h reperfusion, no regional differences in GAPDH activity, reaction Vmax or Km, GAP concentrations or GMR were detectable. CONCLUSIONS: These results suggest that inhibition of GAPDH activity may represent an important point at which glycolysis is limited during reperfusion, and further, that the mechanisms of enzyme inhibition do not involve simple oxidation or S-thiolation of critical active site thiol groups.

Animals↗

Oxidative stress in Huntington's disease.

It has been five years since the elucidation of the genetic mutation underlying the pathogenesis of Huntington's disease (HD) (97), however the precise mechanism of the selective neuronal death it propagates still remains an enigma. Several different etiological processes may play roles, and strong evidence from studies in both humans and animal models suggests the involvement of energy metabolism dysfunction, excitotoxic processes, and oxidative stress. Importantly, the recent development of transgenic mouse models of HD led to the identification of neuronal intranuclear inclusion bodies in affected brain regions in both mouse models and in HD brain, consisting of protein aggregates containing fragments of mutant huntingtin protein. These observations opened new avenues of investigation into possible huntingtin protein interactions and their putative pathogenetic sequelae. Amongst these studies, findings of elevated levels of oxidative damage products such as malondialdehyde, 8-hydroxydeoxyguanosine, 3-nitrotyrosine and heme oxygenase in areas of degeneration in HD brain, and of increased free radical production in animal models, indicate the involvement of oxidative stress either as a causative event, or as a secondary constituent of the cell death cascade in the disease. Here we review the evidence for oxidative damage and potential mechanisms of neuronal death in HD.

Animals↗

TNF-&#x3b1;/NF-&#x3ba;B mediated upregulation of Dectin-1 in hyperglycemic obesity: implications for metabolic inflammation and diabetes.

BACKGROUND: Dectin-1, a key innate immune receptor, plays a critical role in cellular responses and is implicated in chronic inflammation and metabolic syndromes. This study addresses a pivotal gap in elucidating the regulatory mechanism governing Dectin-1 expressionin obesity and diabetes, hypothesizing that hyperglycemia and TNF-&#x3b1; synergistically upregulate Dectin-1 in adipose tissue (AT), thereby exacerbating inflammatory responses and contributing to metabolic dysfunction. METHODS: The study included 95 overweight and obese Kuwaiti individuals, categorized into prediabetic (HbA1c&#x2009;<&#x2009;6.5%) and diabetic (HbA1c&#x2009;&#x2265;&#x2009;6.5%) groups. Anthropometric and clinical measurements were recorded. AT biopsies were obtained for RNA extraction and immunohistochemistry. Pre-adipocytes from lean and obese individuals were cultured, differentiated into adipocytes, and treated with TNF-&#x3b1; under normal or high-glucose conditions to assess Dectin-1 expression. Chromatin immunoprecipitation (ChIP) assays analyzed NF-&#x3ba;B binding to the Dectin-1 promoter. Wildtype and TNF-&#x3b1;-/- mice were used to evaluate TNF-&#x3b1;'s effect on Dectin-1 expression in AT. RESULTS: Our data demonstrate that hyperglycemic obesity significantly induces Dectin-1 expression in AT through the TNF-&#x3b1;/NF-&#x3ba;B signaling pathway. In a cohort of 95 obese individuals, subdivided into prediabetics (HbA1c&#x2009;<&#x2009;6.5%, n&#x2009;=&#x2009;49) and diabetics (HbA1c&#x2009;&#x2265;&#x2009;6.5%, n&#x2009;=&#x2009;46), a strong positive correlation was observed between AT Dectin-1 transcripts and plasma HbA1c levels exclusively in diabetic participants, underscoring the specificity of Dectin-1 upregulation in hyperglycemic conditions. Elevated Dectin-1 expression was consistently associated to increased inflammation markers. Immunohistochemical analysis revealed co-localization and concurrent upregulation of Dectin-1 and TNF-&#x3b1; proteins in hyperglycemic AT. Functional assays in TNF-&#x3b1; deficient mice and human adipocytes further validated that TNF-&#x3b1; and hyperglycemia act cooperatively to regulate Dectin-1 expression. Mechanistically, we demonstrated that NF-&#x3ba;B directly binds to the Dectin-1 promoter, mediating its transcriptional activation in response to glucose and TNF-&#x3b1;. CONCLUSION: This study significantly advances the understanding of upregulation Dectin-1 in metabolic inflammation, filling a crucial niche in diabetes research and suggesting new therapeutic targets for obesity-related metabolic disorders.

Humans↗

How Does Tendon Region, Donor, and the Presence of Disease Affect Protein Composition of the Achilles Tendon?

BACKGROUND: Response to treatment for tendinopathy is variable, which may reflect variability in underlying etiology and capacity for the tendon to respond to treatment. Understanding variability in tendon protein composition may help improve our understanding of the mechanistic underpinnings of painful tendon degeneration and inform treatment targets. QUESTIONS/PURPOSES: (1) What factors (tendon region, individual characteristics, presence of disease) contribute to protein compositional (proteomic) and structural variation in human Achilles tendons? (2) What compositional changes characterize tendinopathy, and what protein interactions might contribute to tendon degeneration? (3) How does diabetes influence tendon composition, and what mechanisms might underlie tendon dysfunction in individuals with diabetes? METHODS: In this exploratory, cross-sectional study, human Achilles tendon specimens were obtained from individuals with (diabetes group, n = 5) or without diabetes (control group, n = 5) undergoing lower extremity amputation and from individuals undergoing tendon debridement surgeries for tendinopathy (tendinopathy group, n = 8). Specimens were collected between 2019 and 2023. Protein abundances were quantified and analyzed using mass spectrometry, hierarchical clustering, and principal component analysis. To evaluate the role of tendon region and donor on tendon protein compositional variability, we assessed proteomic differences between three regions in nontendinopathic tendons from three individuals. To identify the contribution of disease (that is, presence of tendinopathy or diabetes) on protein composition, we compared tendons from the tendinopathy (n = 8 [2 males, 6 females], mean &#xb1; SD age 48 &#xb1; 11 years), diabetes (n = 5 [3 males, 2 females], age 54 &#xb1; 9 years), and control (n = 5 [3 males, 2 females], age 42 &#xb1; 12 years) groups. Proteomic differences associated with tendinopathy and diabetes were further examined using functional enrichment and protein-protein interaction network analysis. RESULTS: Variability in tendon protein composition was primarily from presence of disease, followed by donor and then tendon region. Protein composition distinguished tendons with tendinopathy from controls, with 311 proteins differentially expressed (152 overexpressed and 159 underexpressed; fold change &#x2265; 1.5, p < 0.05) and higher Bonar scores indicating greater degeneration (mean &#xb1; SD Bonar score tendinopathy group 8.6 &#xb1; 1.2 versus control group 2.1 &#xb1; 0.7; p = 0.01). Pathway analysis identified dysregulation in extracellular matrix remodeling (TIMP1, MMP3, MMP10), inflammatory response (TNF-&#x3b1;, EGFR1), and metabolic reprogramming. Tendons from individuals with diabetes exhibited minimal proteomic changes compared with the control group, with 66 differentially expressed proteins (31 overexpressed and 35 underexpressed; fold change &#x2265; 1.5, p < 0.05) with no histopathologic differences between diabetes and control group tendons (mean &#xb1; SD Bonar score diabetes group 3.4 &#xb1; 1.0 versus control group 2.1 &#xb1; 0.7; p = 0.19). Tendons in the diabetes group showed reductions in Type I collagen, enrichment of pathways associated with fibrosis and metabolic dysfunction, and inflammatory pathways associated with &#x3b1; 6 &#x3b2; 4 integrin. CONCLUSION: Our findings indicate that Achilles tendon composition primarily differs based on disease etiology, with tendinopathy showing extensive extracellular matrix disruption and inflammatory activity, whereas tendons from individuals with diabetes exhibit more subtle compositional changes. This distinction suggests that tendinopathy may require targeted interventions addressing tissue remodeling and inflammation, whereas diabetes may predispose tendons to injury but not directly result in degeneration. Understanding these protein compositional variations can help refine hypotheses about disease progression, treatment response, and potential therapeutic targets. CLINICAL RELEVANCE: While proteomic analysis is not currently a part of routine clinical assessment, these findings provide a framework for identifying protein markers that may aid in early diagnosis or patient stratification to improve treatment alignment. Future studies could determine whether these proteomic changes correlate with treatment response and further inform our understanding of early-stage degeneration from chronic disease. By bridging molecular findings with clinical presentation, this study lays the groundwork for future research on precision medicine approaches for tendon disorders, with the long-term goal of tailoring treatment based on both biological and symptomatic characteristics.

Humans↗

Dissociation between exertional symptoms and circulatory function in patients with heart failure.

BACKGROUND: Patients with heart failure frequently report exertional dyspnea and fatigue. These symptoms are usually attributed to circulatory dysfunction and therefore are typically treated with cardiovascular medications. Serial assessment of exertional symptoms has also become the principal method used to assess drug efficacy in heart failure. Nevertheless, the relation between exertional symptoms in heart failure and circulatory dysfunction remains uncertain. METHODS AND RESULTS: This study was undertaken to investigate the relation between exertional symptoms, ventilatory and skeletal muscle dysfunction, and circulatory function in patients with heart failure. To this end, 52 ambulatory patients with heart failure underwent hemodynamic monitoring during maximal treadmill exercise testing. During exercise, the severity of dyspnea and fatigue was evaluated on a scale of 6 to 20 (Borg scale). The level of perceived exercise intolerance during daily activities was evaluated with the Minnesota Living With Heart Failure Questionnaire and the Yale Dyspnea-Fatigue Index. Maximal treadmill exercise increased the VO2 to 13.4 +/- 2.8 mL.min-1.kg-1, the dyspnea score to 15.7 +/- 2.3, the fatigue score to 14.8 +/- 3.4, the pulmonary wedge pressure to 28 +/- 11 mm Hg, and the pulmonary artery lactate concentration to 34.5 +/- 16.3 mg/dL and decreased the pulmonary artery hemoglobin oxygen saturation to 30 +/- 9%. The level of perceived dyspnea had no relation to the pulmonary wedge pressure and correlated only minimally with the level of excessive ventilation (r = 39). The level of perceived fatigue correlated only weakly with blood lactate concentration (r = .55). Eleven patients (21%) exhibited a normal cardiac output and wedge pressure < 20 mm Hg during exercise, 22 (42%) exhibited a normal cardiac output but wedge pressure > 20 mm Hg during exercise, and 19 (37%) exhibited reduced cardiac output and wedge pressure > 20 mm Hg during exercise. Despite these markedly different hemodynamic responses, all three groups exhibited similar levels of fatigue and dyspnea at comparable workloads and had comparable total scores for the Minnesota Living With Heart Failure Questionnaire and the Yale Dyspnea-Fatigue Index. There was no relation between the Living With Heart Failure Questionnaire and peak exercise VO2 and only a weak correlation between the Dyspnea-Fatigue Index and peak VO2 (r = .48). CONCLUSIONS: The level of exercise intolerance perceived by patients with heart failure has little or no relation to objective measures of circulatory, ventilatory, or metabolic dysfunction during exercise. In patients who report severe exertional symptoms, it may be desirable to directly measure hemodynamic response to exercise to ensure that these symptoms are due to circulatory dysfunction.

Adult↗

Ex vivo effect of insulin on normal and diabetic rat hearts hypoperfused with norepinephrine.

The effect of ex vivo insulin on contractile and energy metabolism dysfunctions was examined during hypoperfusion (0.6 ml/min per g heart weight) with 10(-6) M norepinephrine in isolated non-diabetic and streptozotocin-diabetic rats hearts. Insulin (2 mU/min per g heart weight) was infused for 15 min before as well as during 60-min hypoperfusion. Insulin significantly reduced the elevated diastolic tension in diabetic hearts (from 3.8 to 0.7 delta g), but not in non-diabetic hearts (from 1.4 to 1.2 delta g). Insulin partly improved the ATP decrease in the subendocardium of the left ventricle of the diabetic hearts (from 3.5 to 10.2 mumol/g dry weight) but did not affect non-diabetic hearts (from 6.9 to 6.8 mumol/g dry weight). Insulin also partly improved the creatine phosphate decrease and the inorganic phosphate increase in diabetic hearts only. Lactate accumulation was greater in non-diabetic than in diabetic hearts, even in the presence of insulin (77 vs. 45 mumol/g dry weight). The results indicate that acute intracoronary application of insulin in diabetic hearts improves hypoperfusion with norepinephrine injury to a level above that of non-diabetic hearts, but does not improve a less severe injury in non-diabetic hearts.

Animals↗

The electroencephalogram in altered states of consciousness.

The EEG can be very helpful in the evaluation of patients with altered states of consciousness. Diffuse slowing of background rhythms and the presence of triphasic waves suggests metabolic dysfunction, particularly hepatic. Generalized fast activity may be seen in patients with a drug intoxication. Abnormalities, such as PLEDs or focal continual PDA, support a diagnosis of supratentorial lesion, whereas a normal appearing EEG in a comatose patient suggests a brain-stem lesion. In addition, the EEG may reveal that the alteration in consciousness is due to continual epileptic activity without motor manifestations (nonconvulsive status) that had not been suspected. As indicated, certain patterns have prognostic implications. However, as these patterns are not specific for a single etiology, the EEG is of most help when the cause is known. For example, burst suppression or a diffuse alpha pattern coma can be seen in either hypoxia or a drug intoxication. With hypoxia, these patterns carry an extremely poor prognosis for useful recovery, whereas complete recovery is often seen in cases secondary to drug ingestion. As a more extreme example, a patient whose EEG shows ECS secondary to drug intoxication may recover but will not if the ECS is due to hypoxia or severe head trauma. If the etiology of the encephalopathy is unknown, then sequential records are needed for prognosis.

Adolescent↗

Effects of tadenan pretreatment on bladder physiology and biochemistry following partial outlet obstruction.

PURPOSE: Tadenan is a pharmaceutical agent used in the treatment of BPH. Prior studies demonstrated that pretreatment of rabbits with Tadenan significantly reduced the contractile dysfunction following two weeks of partial outlet obstruction. The specific aim of the present study was to determine the effect of Tadenan pretreatment on the time course of the response to partial outlet obstruction and correlate the effect of Tadenan on the contractile responses to field stimulation, bethanechol, and KCl with both mitochondrial enzyme activity (citrate synthase) and sarcoplasmic reticular function (calcium-ATP'ase activity). MATERIALS AND METHODS: Sixty male New Zealands white rabbit (3 to 5 kg.) were separated into 12 groups of 5 rabbits each. Each rabbit in groups 1-6 received Tadenan orally at 100 mg./kg./day for three weeks; each rabbit in groups 7-12 received vehicle (peanut oil). Each rabbit in groups 2-6 and 8-12 received a partial outlet obstruction as described below. One group of Tadenan treated and one group of vehicle-treated rabbits were euthanized at 1, 3, 5, 7, and 14 days following partial outlet obstruction. The non-obstructed groups were studied after 4 weeks of drug or vehicle treatment. Each bladder was rapidly removed and weighed, and 3 longitudinal strips prepared and mounted in individual baths for contractile studies. The remainder of the bladder was frozen for biochemical analysis. The contractile responses to field stimulation, bethanechol, and KCl were determined; and the enzyme activities of citrate synthase (marker for mitochondrial function) and calcium-ATP'ase (marker for sarcoplasmic reticulum) were determined. RESULTS: 1) Tadenan did not reduce the effect of partial outlet obstruction on bladder mass. 2) Although the contractile responses to forms of stimulation were reduced at 1 day following partial outlet obstruction, Tadenan pretreatment resulted in a significant protective effect on the contractile responses to field stimulation, bethanechol, and KCl at 3, 5, 7, and 14 days of obstruction. 3) The activities of both citrate synthase and calcium ATP'ase were reduced significantly at 1 day following obstruction for both Tadenan treated and vehicle treated groups. The activities of both enzymes returned to near normal levels at 7 and 14 days for the Tadenan groups whereas the activities of both enzymes remained significantly reduced in the vehicle treated groups. CONCLUSIONS: These results clearly demonstrate that Tadenan pretreatment protected the bladder from both the contractile and metabolic dysfunctions induced by partial outlet obstruction.

Adenosine Triphosphate↗

Transcriptome Analysis and Experimental Validation of Palmitoylation- Related Biomarkers in Atherosclerosis.

INTRODUCTION: Protein palmitoylation contributes to membrane localisation, signal transduction, and cell-fate regulation. It is closely associated with lipid metabolic dysfunction, immune inflammation, and vascular remodelling in atherosclerosis (AS). However, key palmitoylation-related transcriptomic markers and their potential causal associations with AS remain incompletely defined. METHODS: The Gene Expression Omnibus (GEO) dataset GSE100927 was used as the training cohort, and GSE43292 was used as an external validation cohort. Differentially expressed genes were identified using limma and intersected with palmitoylation-related genes to obtain palmitoylation-related differentially expressed genes (PRDEGs). Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) enrichment analyses were then performed using clusterProfiler. Two-sample Mendelian randomisation was used to evaluate potential causal relationships between characteristic genes and AS. Feature selection was conducted using random forest and support vector machine recursive feature elimination (SVM-RFE), and the overlapping genes selected by both methods were retained. Receiver operating characteristic (ROC) curves were used to assess diagnostic performance. A five-gene nomogram was constructed, and its clinical utility was evaluated using calibration curves and decision curve analysis (DCA). Gene set variation analysis (GSVA) was applied to compare pathway activity between high- and low-expression groups for each core gene. Single-cell analysis using Seurat and expression-based cell-cell communication analysis using CellChat were conducted with GSE159677, and upstream transcription factors were predicted using NetworkAnalyst. For in vivo validation, an AS model was established in ApoE&#x2078;/&#x2078; mice fed a high-fat diet, and aortic gene and protein expression were assessed by RT-qPCR and western blotting. RESULTS: In GSE100927, 51 PRDEGs were identified. GO and KEGG enrichment analyses highlighted pathways associated with regulation of monoatomic ion transport, sarcomere and myofibril organisation, and immune inflammation. Mendelian randomisation suggested a potential protective causal association between SLC7A7 and AS. By integrating MR with random forest and SVM-RFE feature selection, we prioritised five core genes: PLCB2, GMIP, NEXN, PLN, and SLC7A7. These genes showed good diagnostic performance in GSE43292. The resulting nomogram was well calibrated and demonstrated stable net benefit in decision curve and clinical impact curve analyses. Single-gene GSVA identified consistently activated pathways across multiple genes, including innate and adaptive immune recognition, calcium signalling and myocardial contraction/cardiomyopathy, extracellular matrix-receptor interaction, cell junction pathways, autophagy-lysosome pathways, and several metabolic programmes. At the single-cell level, PLCB2 and GMIP were predominantly expressed in T cells and macrophages, NEXN and PLN were enriched in vascular smooth muscle cells, and SLC7A7 was mainly expressed in macrophages. CellChat analysis indicated increased signals for immune-related ligand-receptor interactions. In ApoE&#x2078;/&#x2078; mice fed a high-fat diet, PLCB2, GMIP, and SLC7A7 were upregulated, whereas NEXN and PLN were downregulated; protein-level changes were concordant with the transcriptomic trends. DISCUSSION: These findings indicate that palmitoylation-related dysregulation in AS converges on immune inflammation, calcium signalling/contractile programmes, ECM remodelling, and autophagy-linked metabolism. The five-gene panel is supported by external validation, single-cell localisation to immune and vascular compartments, and concordant results in ApoE&#x2078;/&#x2078; mice. CONCLUSION: This study identified and validated five palmitoylation-related genes associated with AS. SLC7A7 showed a potential protective causal signal in MR analysis. The enriched pathway patterns linked these genes to immune inflammation, calcium signalling-contraction coupling, ECM remodelling, cell adhesion, and autophagy- associated metabolic reprogramming. The five-gene nomogram showed potential utility for diagnostic classification and decision support, nominating candidate biomarkers and pathway targets for AS molecular subtyping, diagnosis, and mechanistic investigation.

Atherosclerosis (AS)↗

Familial progressive supranuclear palsy: detection of subclinical cases using 18F-dopa and 18fluorodeoxyglucose positron emission tomography.

BACKGROUND: Progressive supranuclear palsy (PSP) is generally considered to be a sporadic disease; however, occasional cases of familial PSP have been described. The rarity of reports of familial PSP may be attributed in part to an inability to detect subclinical disease in affected relatives who subsequently die before symptoms clinically develop. OBJECTIVE: To study regional cerebral dopaminergic function and glucose metabolism in members of 2 large kindreds with familial PSP to identify subclinical cases. METHODS: Three clinically affected members from the 2 PSP kindreds were scanned with both (18)F-dopa and (18)fluorodeoxyglucose ((18)FDG) positron emission tomography (PET). Fifteen asymptomatic first-degree relatives were scanned with (18)F-dopa PET; 10 of them also underwent a second PET study with (18)FDG. RESULTS: All 3 clinically affected PSP patients showed a significant reduction in caudate and putamen (18)F-dopa uptake along with a significant reduction in striatal, lateral, and medial premotor area and dorsal prefrontal cortex glucose metabolism. In 4 of the 15 asymptomatic relatives, caudate and putamen (18)F-dopa uptake was 2.5 SDs lower than the normal mean. These 4 subjects and a fifth asymptomatic relative with normal (18)F-dopa uptake showed a significant reduction of cortical and striatal glucose metabolism in a pattern similar to that of their affected relatives. CONCLUSION: (18)F-dopa and (18)FDG PET allowed us to identify 5 cases with subclinical metabolic dysfunction among 15 subjects (33%) at risk for PSP, suggesting that this approach is useful for characterizing the pattern of aggregation in PSP kindreds.

Adult↗

Ocular histopathology and ultrastructure of Sanfilippo's syndrome, type III-B.

The ocular histopathology of systemic mucopolysaccharidosis, type III-B (Sanfilippo's syndrome) was studied using histochemical and ultrastructural techniques. Cytoplasmic, single-membrane-bound vacuoles containing the major storage product, acid mucopolysaccharide, were found in virtually every ocular tissue. Lamellar cytoplasmic membranous bodies of complex lipid were found mainly in the retinal ganglion cells and the lens epithelium. Many tissues had inclusions that were of an intermediate type and were composed of combined fibrillogranular and lamellar membranous material. Hypopigmentation of the neuroepithelial pigment layers (ie, iris, ciliary, and retinal) seems to be the result of autophagocytosis with melanolysis. Photoreceptor cell degeneration was similar to that seen in some forms of retinitis pigmentosa. The mechanism of photoreceptor cell degeneration is unknown. It may be the result of metabolic dysfunction due to accumulation of mucopolysaccharide in the retinal pigment epithelium.

Adolescent↗

The expanding clinical phenotype of the tRNA(Leu(UUR)) A-->G mutation at np 3243 of mitochondrial DNA: diabetic embryopathy associated with mitochondrial cytopathy.

We describe a family which demonstrates and expands the extreme clinical variability now known to be associated with the A-->G transition at nucleotide position 3243 of the mitochondrial DNA. The propositus presented at birth with clinical manifestations consistent with diabetic embryopathy including anal atresia, caudal dysgenesis, and multicystic dysplastic kidneys. His co-twin was normal at birth, but at 3 months of life, presented with intractable seizures later associated with developmental delay. The twins' mother developed diabetes mellitus type I at the age of 20 years and gastrointestinal problems at 22 years. Since age 19 years, the maternal aunt has had recurrent strokes, seizures, mental deterioration and deafness, later diagnosed as MELAS syndrome due to the tRNA(Leu(UUR)) A-->G mutation. A maternal uncle had diabetes mellitus type I, deafness, and normal intellect, and died at 35 years after recurrent strokes. This pedigree expands the known clinical phenotype associated with tRNA(Leu(UUR)) A-->G mutation and raises the possibility that, in some cases, diabetic embryopathy may be due to a mitochondrial cytopathy that affects both the mother's pancreas (and results in diabetes mellitus and the metabolic dysfunction associated with it) and the embryonic/fetal and placental tissues which make the embryo more vulnerable to this insult.

Abnormalities, Multiple↗