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Population-scale detection of methylation outliers from long-read genome sequencing.

BACKGROUND: Aberrant DNA methylation can mediate the functional effects of rare genetic variation and contribute to imprinting disorders, repeat expansion diseases, and other pathogenic regulatory mechanisms. Long-read sequencing technologies now enable genome-wide detection of CpG methylation alongside genetic variation from a single assay. However, methods for systematic identification and interpretation of methylation outliers from long-read sequencing data remain limited. METHODS: We developed METAFORA, a computational workflow for detecting methylation outlier regions from PacBio and Oxford Nanopore long-read sequencing data. METAFORA constructs population-level methylation references, segments the genome into correlated CpG blocks, infers technical and biological sources of variation through hidden factor estimation, models uncertainty due to variable depth sequencing, and computes covariate-adjusted methylation outlier scores for individual samples. We applied METAFORA across large long-read sequencing cohorts and integrated methylation outliers with multi-omic data. METAFORA is implemented as a snakemake workflow available at https://github.com/tjense25/METAFORA. RESULTS: METAFORA identified methylation outlier regions associated with rare structural variants, tandem repeat expansions, and imprinting abnormalities. We found outlier regions were enriched for molecular outliers across transcriptomic and chromatin accessibility datasets, supporting their functional relevance in gene regulation. In a representative case, METAFORA identified an imprinting defect affecting the GNAS locus associated with an STX16 deletion. CONCLUSIONS: METAFORA enables scalable detection and interpretation of methylation outliers from long-read sequencing data and provides a framework for integrating epigenetic outliers with genomic and multi-omic analyses. These approaches may improve interpretation of rare regulatory variation and support discovery of clinically relevant epigenetic abnormalities in genomic medicine.

DNA methylation↗

A relationship between gap junction-mediated intercellular communication and the in vitro developmental capacity of murine embryonic stem cells.

Two metabolic cooperation-deficient variants, 1P9 and 2P2[1], have been isolated from the embryonic stem cell line B2B2. Characterization of these cell lines has shown that both variants are severely restricted with respect to embryoid body differentiation capacity while a revertant isolated from 1P9, designated 2H4, has its differentiation phenotype restored to a level comparable with that of the parent line. These results are interpreted as indicating that the metabolic cooperation deficiency and the restricted differentiation phenotype of 1P9 are causally related. A revertant isolated from 2P2[1], designated H19, remains severely restricted with regard to embryoid body differentiation, suggesting that in these lines a secondary event deleterious to differentiation, but unrelated to metabolic cooperation, has taken place.

Animals↗

Heparitin sulfates (heparan sulfates) of atherosclerosis-susceptible and atherosclerosis-resistant pigeon aortas.

Electrophoretic mobilities of heparitin sulfates (HS) from the thoracic aorta and the celiac bifurcation of atherosclerosis-susceptible White Carneau and atherosclerosis-resistant Show Racer pigeons at four ages were examined. Atherosclerosis-susceptible White Carneau aortas contain, in addition to their normal HS component, a variant type of HS (HS') in pre, early- and moderately-atherosclerotic celiac foci which is not found in the thoracic regions of either breed or in Show Racer celiac foci at any age. These findings are interpreted to suggest a role of HS' in the pathogenesis of atherosclerosis and to propose an explanation for atherosclerotic susceptibility and resistance in pigeons.

Age Factors↗

Patient-related pitfalls and artifacts in nuclear medicine imaging.

Quality control in nuclear medicine is all important. This applies not only to preparation of the patient and acquisition of the image, but also to interpretation of the study. Although it may seem self-evident, it is important to remain aware of artifacts that are directly related to the patient and need special consideration. Furthermore, at times the distinction between normal variants and artifacts can be difficult. Commonly encountered patient-related artifacts include artifacts caused by attenuation, contamination artifacts, and artifacts caused by intravenous lines, tubes, and catheters. Less commonly, artifacts arise because of the use of multiple isotopes, the presence of fistulas or surgically altered anatomy, and pharmaceuticals and other substances interfering with expected radiopharmaceutical uptake and distribution. The diagnostic accuracy of nuclear medicine reporting can be improved by awareness of these patient-related artifacts. Both awareness and experience are also important when it comes to detecting and identifying normal (and abnormal) variants.

Artifacts↗

Voltage dependence of Na-Ca exchanger conformational currents.

Properties of a transient current (Icont) believed to reflect a conformational change of the Na-Ca exchanger molecules after Ca2+ binding were investigated. Intracellular Ca2+ concentration jumps in isolated cardiac myocytes were generated with flash photolysis of caged Ca2+ dimethoxynitrophenamine, and membrane currents were simultaneously measured using the whole-cell variant of the patch-clamp technique. A previously unresolved shallow voltage dependence of Icont was revealed after developing an experimental protocol designed to compensate for the photoconsumption of the caged compound. This voltage dependence can be interpreted to reflect the distribution of Na-Ca exchanger conformational states with the Ca2+ binding site exposed to the inside of the cell immediately before the flash. Analysis performed by fitting a Boltzmann distribution to the observed data suggests that under control conditions most exchanger molecules reside in states with the Ca2+ binding site facing the outside of the cell. Dialysis of the cytosol with 3',4'-dichlorobenzamil, an organic inhibitor of the Na-Ca exchange, increased the magnitude of Icont and changed the voltage dependence, consistent with a parallel shift of the charge/voltage curve. This shift may result from intracellular DCB interfering with an Na(+)-binding or Na(+)-translocating step. These observations are consistent with Icont arising from a charge movement mediated by the Na-Ca exchanger molecules after binding of Ca2+.

Animals↗

Implementation and uses of automated de novo peptide sequencing by tandem mass spectrometry.

There are several computer programs that can match peptide tandem mass spectrometry data to their exactly corresponding database sequences, and in most protein identification projects, these programs are utilized in the early stages of data interpretation. However, situations frequently arise where tandem mass spectral data cannot be correlated with any database sequences. In these cases, the unmatched data could be due to peptides derived from novel proteins, allelic or species-derived variants of known proteins, or posttranslational or chemical modifications. Two additional problems are frequently encountered in high-throughput protein identification. First, it is difficult to quickly sift through large amounts of data to identify those spectra that, due to poor signal or contaminants, can be ignored. Second, it is important to find incorrect database matches (false positives). We have chosen to address these difficulties by performing automatic de novo sequencing using a computer program called Lutefisk. Sequence candidates obtained are used as input in a homology-based database search program called CIDentify to identify variants of known proteins. Comparison of database-derived sequences with de novo sequences allows for electronic validation of database matches even if the latter are not completely correct. Modifications to the original Lutefisk program have been implemented to handle data obtained from triple quadrupole, ion trap, and quadrupole/time-of-flight hybrid (Qtof) mass spectrometers. For example, the linearity of mass errors due to temperature-dependent expansion of the flight tube in a Qtof was exploited such that isobaric amino acids (glutamine/lysine and oxidized methionine/ phenylalanine) can be differentiated without careful attention to mass calibration.

Amino Acid Sequence↗

Y25S variant of Paracoccus pantotrophus cytochrome cd1 provides insight into anion binding by d1 heme and a rare example of a critical difference between solution and crystal structures.

Tyr25 is a ligand to the active site d1 heme in as isolated, oxidized cytochrome cd1 nitrite reductase from Paracoccus pantotrophus. This form of the enzyme requires reductive activation, a process that involves not only displacement of Tyr25 from the d1 heme but also switching of the ligands at the c heme from bis-histidinyl to His/Met. A Y25S variant retains this bis-histidinyl coordination in the crystal of the oxidized state that has sulfate bound to the d1 heme iron. This Y25S form of the enzyme does not require reductive activation, an observation previously interpreted as meaning that the presence of the phenolate oxygen of Tyr25 is the critical determinant of the requirement for activation. This interpretation now needs re-evaluation because, unexpectedly, the oxidized as prepared Y25S protein, unlike the wild type, has different heme iron ligands in solution at room temperature, as judged by magnetic circular dichroism and electron spin resonance spectroscopies, than in the crystal. In addition, the binding of nitrite and cyanide to oxidized Y25S cytochrome cd1 is markedly different from the wild type enzyme, thus providing insight into the affinity of the oxidized d1 heme ring for anions in the absence of the steric barrier presented by Tyr25.

Anions↗

Limits to causal inference based on Mendelian randomization: a comparison with randomized controlled trials.

"Mendelian randomization" refers to the random assortment of genes transferred from parent to offspring at the time of gamete formation. This process has been compared to a randomized controlled trial of genetic variants. This could greatly aid observational epidemiology by potentially allowing an unbiased estimate of the effects of gene products on disease outcomes. However, studies utilizing Mendelian randomization to estimate effects of gene products on outcomes should be interpreted with caution. In this paper, the authors discuss some of the challenges facing epidemiologists in the analysis and interpretation of Mendelian randomization studies, particularly those that become apparent when the analogy with randomized controlled trials is closely examined. The authors conclude that Mendelian randomization is a powerful addition to etiologic research tools. However, care must be taken, because drawing valid causal inferences from its application depends upon more extensive assumptions than are required in randomized controlled trials.

Causality↗

Prefrontal cortex and dynamic categorization tasks: representational organization and neuromodulatory control.

We present a computational model of the intradimensional/ extradimensional (ID/ED) task (a variant of the Wisconsin card sorting task) that simulates the performance of intact and frontally lesioned monkeys on three different kinds of rule changes (Dias et al., 1997, J Neurosci 17:9285-9297). Although Dias et al. interpret the lesion data as supporting a model in which prefrontal cortex is organized into different processing functions, our model suggests an alternative account based on representational content. A key aspect of the model is that prefrontal cortex representations are organized according to different levels of abstraction, with orbital areas encoding more specific featural information and dorsolateral areas encoding more abstract dimensional information. This representational scheme of the model is integrated with two additional key elements: (i) activation-based working memory representations controlled by a dynamic gating mechanism that simulates the hypothesized phasic actions of dopaminergic neuromodulation in prefrontal cortex, which acts to stabilize or destabilize frontal representations based on success in the task; and (ii) a weight-based associative learning system simulating posterior cortex and other subcortical areas, where the stimulus-response mappings are encoded. Frontal cortex contributes to the task via top-down activation-based biasing of task-appropriate features and dimensions in this posterior cortex system - this top-down biasing is specifically important for overcoming prepotent associations after a sorting rule reverses. The ability of the model to capture the double-dissociation observed by Dias et al. with orbital versus dorsolateral lesions supports the validity of these principles, many of which have also been useful in accounting for other frontal phenomena.

Animals↗

Polymorphisms in XPD exons 10 and 23 and bladder cancer risk.

INTRODUCTION: The nucleotide excision repair gene, xeroderma pigmentosum complementation group D (XPD), has been hypothesized to have a role in cancer risk, but results from prior molecular epidemiologic studies and genotype-phenotype analyses are conflicting. MATERIALS AND METHODS: We examined the frequency of the XPD Asp312Asn polymorphism in exon 10 and the XPD Lys751Gln polymorphism in exon 23 in 505 incident bladder cancer cases and 486 healthy controls. RESULTS: Overall, the XPD exon 10 and 23 genotypes were not associated with bladder cancer risk, after adjusting for age, sex, ethnicity, and smoking status. A gender-specific role was evident that showed an increased risk for women, but not for men, associated with the variant genotypes for both exons. For example, when the exon 23 variant allele genotypes were combined (Lys/Gln + Gln/Gln), there was an increased bladder cancer risk in women [odds ratio (OR), 1.69; 95% confidence interval (95% CI), 1.12-2.58] but not in men (OR, 0.99; 95% CI, 0.79-1.24; P(interaction) = 0.041; OR, 1.62; 95% CI, 1.02-2.58). There was also a gene-smoking interaction that showed the variant alleles for either exon or the combination of both increase the risk of bladder cancer for light and heavy smokers. For exon 23 (P(interaction) = 0.057; OR, 1.21; 95% CI, 0.99-1.47), heavy smokers (> or = 20 pack-years) who carried the exon 23 variant allele genotypes had an OR of 4.13 (95% CI, 2.53-6.73), whereas heavy smokers with the wild-type genotypes were at lower risk (OR, 3.55; 95% CI, 2.19-5.75). Moderate smokers (1-19 pack-years) with the variant allele genotypes had an OR of 1.54 (95% CI, 0.94-2.53), whereas moderate smokers with the wild-type genotypes had an OR of 1.12 (95% CI, 0.63-1.98). CONCLUSIONS: Although we did not observe main effects associated with the XPD genotypes, these results do suggest the variant allele genotypes were associated with increased bladder cancer risk in women and smokers with statistically significant interactions in the exon 23 polymorphism. Although there is biological plausibility, these novel findings for gender and smoking should be interpreted with caution upon confirmation in larger studies.

Case-Control Studies↗

Adrenal cortical carcinomas with myxoid differentiation in the domestic ferret (Mustela putorius furo).

A total of 15 adrenocortical carcinomas with myxoid differentiation from 15 ferrets were evaluated in this retrospective study. Six of these ferrets (40%) either were euthanatized or died due to invasive and/or metastatic disease. The myxoid component was a variable part (between 5% and 95%) of the adrenal cortical neoplasm and consisted of sheets and cords of small, polygonal neoplastic cells that formed lumenlike spaces. Such spaces contained a variable amount of alcian blue (pH 2.5)-positive mucinous product (i.e., acidic mucopolysaccharides). Neoplastic cells were negative for the argentaffin reaction, but immunohistochemically they were strongly positive for vimentin and alpha-inhibin and lightly positive for synaptophysin. Proliferating cell nuclear antigen (PCNA)-labeling indices (LI) of adrenal cortical neoplastic cells within the myxoid component of the neoplasm were significantly elevated (P < 0.05) compared with those of typical neoplastic adrenal cortical cells or the adjacent nonneoplastic zona reticularis. Ultrastructurally, cells in the myxoid component exhibited a typical adrenocortical phenotype characterized by cytoplasmic lipid vacuoles, prominent rough and smooth endoplasmic reticulum, and zonula adherens. This lesion was interpreted as an adrenal cortical carcinoma with myxoid differentiation and appeared to be highly malignant based on PCNA LI, rate of invasion into adjacent tissue, and metastasis (6/15). This report is the first description of this histologic variant in the ferret, which morphologically resembled the rare myxoid variant of adrenocortical carcinoma described in humans.

Adrenal Cortex Neoplasms↗

Structuring of perceived interrelations among life events for college students.

We attempted to structure a student's perceived interrelationships among major life events in an hierarchy by the use of the Interpretive Structural Modeling (ISM) method from paired-comparison data. Analysis suggested that, although the perception of the ages at which an individual will experience major life events is strongly influenced by age norm and is less variant among persons, the perception of the interrelationships among life events is more variant.

Adult↗

Multiple single nucleotide polymorphisms on human chromosome 19q13.2-3 associate with risk of Basal cell carcinoma.

In this paper, we present evidence that alleles of several polymorphisms in the chromosomal region 19q13.2-3, encompassing the genes RAI and XPD, are associated with occurrence of basal cell carcinoma in Caucasian Americans. The association of one of these, RAI-intron1, is sufficiently strong to make mass significance unlikely (P = 0.004, chi(2)). We interpret our combined data to indicate that a specific haplotype partly defined by the alleles of three single nucleotide polymorphisms, RAI intron1(G), RAI exon6(T), and XPD exon 6(C), is associated with a protective gene variant in a region spanning from XPD to ERCC1.

Alleles↗

[The ontogenetic approach to chlorophyll fluorescence studies of plant photosynthetic apparatus under stressful conditions].

Based on the analysis of reasons limiting the application of the method of chlorophyll fluorescence induction for estimating the state of the leaf photosynthetic apparatus under prolonged stress, the necessity of the ontogenetic approach consisting in a more exact determination of leaf age was substantiated. A comparison of the calendar and ontogenetic ways of determination of age of cucumber leaves under controlled conditions revealed essential distinctions in the estimation of plant leaf photosynthetic apparatus by the method of chlorophyll fluorescence induction for two variants distinguishing by the cultivation light regime ("white", 400-700 nm, and "red", 600-700 nm). It was shown that, in the case of prolonged effect of the stress factor on the plant, the unambiguity of the interpretation of chlorophyll fluorescence induction parameters in the estimation of the state of their photosynthetic apparatus depends essentially on the choice of the ontogenetic period of leaves of plants being compared and the accuracy of determination of leaf age.

Chlorophyll↗

[A review of the suspension of paired limbs in vertebrates from functional viewpoints].

The functional interpretation is delivered of the principal morphological subdivisions of the paired limbs in the vertebrate animals, that is of the girdles and free parts. To distinguish between those, the functional criteria are suggested. On this basis the variants are considered of suspension of the fore and hind limbs in various groups of Gnathostomata. In the hind limbs the boundary between the girdle and the free part does always pass through the hip joint. In the fore limb the corresponding boundary passes through the shoulder joint in fishes. However, in most tetrapods it shifts proximally due to the release of the scapulocoracoid mobility. This tendency reached its maximum in turtles and viviparous mammals. The main attention is paid to the analysis of the arrangement of the scapulocoracoid suspension in various groups of tetrapods.

Adaptation, Physiological↗

[Schizo-affective psychosis manifested in adolescence].

141 patients with schizoaffective psychosis were observed: 70 patients in the period of the first manifest attack and 71 ones 10-20 years after it (catamnestic group). It was found that juvenile schizoaffective psychosis is clinically heterogenous endogenous disease, presenting in a continuum of forms in the range from the ones close to affective psychosis to the forms closer to shift-like progredient schizophrenia. The course of the psychosis was determined by peculiarities of nonaffective delirious disorders in clinical picture of manifest attacks. 3 main variants of such attacks are recognized: with domination of delusion of imagination (the 1st type); with domination of a delusion of perception (the 2nd type); with domination of a delirium of perception combined with manifestations of acute interpretative delirium (the 3d type). The 1st and 2nd types of manifest attacks correspond to ICD-10 criteria of schizoaffective psychosis. The course and outcome of juvenile schizoaffective psychosis were less favourable than those of analogus psychosis in adult age.

Adolescent↗

Haplotype analysis of the linkage group HLA-A:HAL-B:Bf and its bearing on the interpretation of the linkage disequilibrium.

The analysis of 650 HLA-A:HAL-B:Bf three-factor haplotypes revealed significant associations only between alleles of the very closely linked genes HLA-A and HLA-B, and Bf, respectively. Most striking is the highly significnat association of the rare Bf variant F1 with HLA-B18 and of S1 with HLA-B13, HLA-B14, and HLA-Bw21. Only random allele distributions were observed when considering the somewhat more distant genes HLA-A and Bf or the higher order interaction at all three genes. From these findings it seems likely that the linkage disequilibrium within the MHC is not due to selective forces, but rather due to a short evolutionary period.

Chromosomes, Human, 6-12 and X↗

Afterimages in fly motion vision.

Afterimage-like effects modulate the responses of fly wide-field motion-sensitive cells following adaptation to stationary or slowly moving patterns. The origin of these afterimages is unclear. They have been interpreted as either the result of adaptation in the early visual system or as a direct consequence of the correlation scheme of motion detection. Using a combination of intracellular recording and computer modelling, we find that afterimage-like effects cannot be satisfactorily explained by a simple version of the correlation model previously proposed by Egelhaaf and Borst (J. Opt. Soc. Am. A 6 (1) (1989) 116). We propose a modified variant of the correlation model featuring a short delay filter and temporal high-pass filtering prior to motion correlation. Our model gives superior predictions of afterimage-like effects induced by a range of stimuli. Our model also predicts changes in cells' image step responses following exposure to motion, suggesting that previous experimental evidence for the "shortening delay" theory of motion adaptation (Biol. Cybern. 54 (1986) 223; Visual Neurosci. 14 (4) (1997) 741) should be re-interpreted in terms of afterimage effects.

Adaptation, Physiological↗