Aberrant Splicing Patterns Could Predict Therapy Response in mRCC

Transcriptomic analysis of more than 100 metastatic renal cell carcinomas (mRCC) has revealed key differences in aberrant alternative gene splicing events between treatment responders and nonresponders that could aid prognostication in future.

“In the near term, these findings could help guide treatment selection by identifying patients more likely to respond to targeted therapies or standard immuno-oncology regimens,” said Patrick Pirrotte, PhD, director of the Integrated Mass Spectrometry Shared Resource at TGen and City of Hope, associate professor in TGen’s Early Detection and Prevention Division, and senior author of the paper.

“Longer term, splicing-derived antigens could provide a foundation for more personalized adoptive immunotherapy strategies tailored to the molecular features of an individual patient’s tumor,” he told Inside Precision Medicine.

Pirrotte explained that “alternative splicing [AS] is a fundamental transcriptional mechanism that expands proteomic diversity in normal cells, but aberrant splicing is increasingly recognized as a feature of cancer that can contribute to tumorigenesis, progression, and metastasis.”

His group, and collaborators, have previously demonstrated that aberrant splicing could act as a broadly relevant biomarker across different malignancies, including ovarian cancer and sarcomatoid renal cell carcinoma, but its diagnostic and predictive potential in mRCC remained largely unexplored.

To address this, Pirrotte and team conducted a retrospective analysis on tumor samples from 101 patients with mRCC who received immune checkpoint inhibitor (n=91) and/or targeted (n=77) therapies. Response rates to each of the therapies were 63% and 77%, respectively.

The researchers report in the Journal for ImmunoTherapy of Cancer that they identified 10 AS events that were specific to mRCC. Six of these were intron retention events and four were exon skipping events.

Differential AS analysis identified 461 slicing events that differed between responders and non-responders to immune checkpoint inhibitors and 253 events that differed between targeted therapy responders and non-responders. In both cases, more than 70% of novel AS events among responders involved intron retention.

“Intron retention was the predominant alternative splicing event observed in patients who responded well to therapy,” observed Pirrotte.

“Mechanistically, intron retention occurs when intronic sequences that are normally removed during RNA processing are retained in the mature transcript. This can generate novel amino acid sequences and, in some cases, tumor-associated antigens derived from aberrant splicing,” he explained. “A high intron-retention burden was associated with an immunogenic tumor microenvironment, marked by adaptive immune activation and enriched antigen processing. In simple terms, these cancer-specific splicing errors may help ‘flag’ tumor cells, making them more visible to the immune system.”

The team then investigated whether differentially spliced sequences shared between the immunotherapy and targeted therapy responder cohorts could potentially act as neoantigenic targets.

This revealed that novel peptide-generating AS events in the genes IFFO1 and ZNF692 were highly expressed among the responders. Both genes are known to play a role in tumorigenesis and metastasis in RCC and colorectal cancer. The researchers note that although the specific impact of AS events within these genes is unclear, the resulting neoantigens could play a role in future treatment approaches.

“It is becoming increasingly feasible to identify splicing-derived neoantigens that could be used in personalized immunotherapy approaches, including adoptive cell therapies such as CAR T-cell or tumor-infiltrating lymphocyte therapies,” said Pirrotte. “These strategies are designed to train or redirect a patient’s immune system to recognize tumor-specific antigen signatures. In this case, the targets would be antigens generated by aberrant splicing events, allowing immune cells to selectively recognize and kill cancer cells.”

Finally, the investigators showed that tumors with higher levels of aberrant splicing were more common among therapy responders than nonresponders. This could potentially be used as a biomarker for treatment response.

“Current biomarkers such as PD-L1 expression and microsatellite instability have shown limited and inconsistent predictive value in mRCC,” said Pirrotte. “In contrast, our study identified a significant association between tumor ‘splicing burden’ (the extent of aberrant splicing) and clinical response to therapy. These findings suggest that the tumor transcriptome, particularly splicing dysregulation, may provide a more informative framework for predicting treatment response and personalizing therapy.”

Before assessment of AS can be implemented in routine clinical practice, the core technologies will need further refinement, including clinically validated RNA sequencing workflows, robust computational pipelines for splicing analysis, and clear regulatory and technical frameworks for using the results to guide treatment decisions or develop biologic therapies.

Pirrotte and team are now assembling validation cohorts to confirm their findings in larger patient populations. They are also expanding their work to other cancer types to determine whether aberrant splicing and splicing-derived antigens represent broadly applicable biomarkers and therapeutic targets.

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Factors Predicting Poor Outcomes in Hypertrophic Cardiomyopathy Uncovered

Five factors predicting death or serious complications in hypertrophic cardiomyopathy, a heart condition where the heart muscle becomes abnormally thick, have been uncovered in a study led by the University of Virginia.

“Hypertrophic cardiomyopathy, with a prevalence of one in 500 in the U.S., is the most frequent cause of sudden cardiac death in young individuals,” explain lead author Christopher Kramer, MD, a researcher at University of Virginia Health, in JAMA.

“Although some patients remain asymptomatic, others develop effort intolerance, exertional angina, progressive heart failure, atrial and ventricular arrhythmias, and sudden cardiac death.”

There is some disagreement about how best to predict risk in patients diagnosed with this condition, which is inherited in 60% of cases, with different factors used for assessment in different places and current guidelines focusing on sudden cardiac death risk and not other serious adverse events such as the risk for heart failure.

This study enrolled 2,698 hypertrophic cardiomyopathy patients from 44 sites across North America and Europe between 2014 and 2017 and followed them for an average of 6.9 years. The participants underwent wide ranging tests on enrollment including cardiac magnetic resonance imaging with core laboratory analysis, genetic testing of 36 cardiomyopathy genes, blood biomarker analysis, and detailed clinical assessments. Patients with pre-existing implantable cardioverter defibrillators, often prescribed to patients with this condition to avert sudden cardiac death, were excluded.

Patients were reviewed once a year by telephone, with an average follow-up time of around seven years. Records were reviewed if events occurred during the study.

Overall, 117 events—death, nonfatal sustained ventricular arrhythmias requiring cardioversion or defibrillation, left ventricular assist device implant or heart transplant—occurred in 104 participants during the follow up period.

Five factors were significant predictors of a poor outcome. These included the extent of scarring on the heart measured by imaging, heart muscle size, and heart chamber size with all three predicting worse outcomes with greater measures. The other two factors were history of heart failure and higher levels of a blood protein marker of heart stress, NT-proBNP.

“Current risk prediction guidelines for hypertrophic cardiomyopathy are imperfect, as they predict only sudden cardiac death, and not heart failure or other fatal and nonfatal cardiac adverse events,” said Kramer in a press statement. “This study is a major advance in that it provides evidence that incorporating these additional assessment methods better predicts risk of adverse outcomes.”

The team plan to continue this work and to develop a risk score as well as to seek external validation from independent databases and researchers using similar measures of risk.

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Steroid receptor coactivator-1: integrating steroid hormone signals to regulate brain function and disease

Steroid receptor coactivator-1 (SRC-1), also known as nuclear receptor coactivator-1 (NCOA1), represents the first identified member of the p160 nuclear receptor coactivator family and plays a pivotal role in integrating steroid hormone signals, regulating gene transcription, and maintaining neural homeostasis in the central nervous system (CNS). SRC-1 exhibits region-specific, cell-type-specific, and sexually dimorphic expression patterns in the brain, with prominent distribution in key regions including the hippocampus, cerebral cortex, hypothalamus, and amygdala. Functional studies demonstrate that SRC-1 participates in diverse neural functions such as learning and memory, energy metabolism, emotional regulation, and reproductive behavior through modulation of synaptic plasticity-related genes, neurotrophic factors, and metabolic pathways. Aberrant SRC-1 expression is closely associated with neurodegenerative diseases, autism spectrum disorders, and glioblastoma. This review systematically summarizes the molecular structure, expression characteristics, physiological functions of SRC-1, and its roles in neurological disorders, while discussing its potential applications as a diagnostic biomarker and therapeutic target.

Serum hypocretin, neurofilament heavy chain, and interleukin-1β as combined predictors of sleep disorders following acute ischemic stroke

BackgroundSleep disorders represent a common and impactful complication following acute ischemic stroke (AIS). This study aimed to identify clinical risk factors and evaluate the predictive value of serum hypocretin (Hcrt), neurofilament heavy chain (NfH), and interleukin-1 beta (IL-1β) for post-stroke sleep disorders.MethodsWe conducted a retrospective observational study of 256 patients with AIS. Patients were classified into sleep disorder (n = 161) and non-sleep disorder (n = 95) groups based on their Pittsburgh Sleep Quality Index scores 7 days after stroke onset. Fasting serum levels of Hcrt, NfH, and IL-1β were measured upon admission. We utilized multivariate logistic regression and receiver operating characteristic (ROC) curves to evaluate predictive performance. The combined model was internally validated using 1,000 bootstrap resamples to assess optimism-corrected discriminative performance.ResultsSleep disorders were present in 62.9% of patients. Nine independent risk factors were identified: age ≥ 65 years (OR = 2.059), snoring history (OR = 1.980), prior stroke (OR = 2.036), lower ADL scores (OR = 1.839), higher HAMD (OR = 1.726) and NIHSS scores (OR = 1.677), decreased serum Hcrt (OR = 1.863), elevated NfH (OR = 2.020), and elevated IL-1β (OR = 1.793; all p < 0.05). Individual biomarker AUCs ranged from 0.742 to 0.781, whereas the combined three-biomarker model achieved a significantly superior AUC of 0.874 (sensitivity 88.82%, specificity 71.58%). Bootstrap internal validation yielded a mean optimism-corrected AUC of 0.861 (95% CI: 0.812–0.903), indicating robust model performance with minimal overfitting.ConclusionClinical variables alongside altered levels of Hcrt, NfH, and IL-1β serve as independent predictors of post-stroke sleep disorders. The combined three-biomarker panel, reflecting neuroendocrine dysregulation, axonal injury, and systemic inflammation, demonstrates substantially superior predictive accuracy over individual biomarkers and offers a clinically practical tool for early identification of high-risk patients.

[Articles] The presynaptic protein bassoon is a biofluid biomarker of synaptic pathology in multiple sclerosis

In conclusion, the presynaptic protein BSN can be quantified in plasma and CSF to assess synaptic pathologies. BSN elevation was already detectable at the earliest disease stages and persisted in progressive MS, underscoring continuous neurodegeneration in MS. Measuring synaptic proteins may complement established biomarkers of neuronal injury to enhance our understanding of neurodegeneration in MS.

At-Home Blood Test Screens for Early Dementia

A simple finger-prick blood test at home combined with online cognitive tests can reveal signs of Alzheimer’s disease, providing a convenient way to screen for early dementia.

The postal blood test, outlined in Nature Communications, is used to measure levels of two blood biomarkers linked with cognitive function: phosphorylated tau at amino acid 217 (p-tau217) and Glial Fibrillary Acidic Protein (GFAP).

It could provide a way to screen for dementia at home and act as a triage resource to identify those at risk earlier and tailor treatments more effectively, particularly in remote or unsupervised settings.

“This work raises the potential for screening people for their risk without the need for clinic visits or complex clinical assessments,” said lead researcher Anne Corbett, PhD, from the University of Exeter.

“It would ensure the people at highest risk could be prioritized for monitoring and diagnosis, unlocking the best support and treatment for those that need it most.”

While blood biomarkers are increasingly being used to diagnose Alzheimer’s disease, scalable tools are needed to reach the 99% of individuals with early cognitive impairment who are not seen in specialist healthcare services.

In an attempt to develop these further, Corbett and team conducted a study involving 174 people, of whom 146 had normal cognition and 28 had dementia.

All were participants in the PROTECT study, a larger investigation of more than 30,000 adults that aims to understand how healthy brains age and why people develop dementia.

Blood samples were collected at home using self-administered capillary blood tests, which were sent for p-tau 217 and GFAP lab testing. Venous blood samples were also available for 40 patients.

p-tau217 has previously been highly accurate at detecting Alzheimer’s disease pathology and is approved by U.S. regulators for symptomatic patients undergoing investigation for cognitive complaints.

GFAP is associated with broader cognitive decline and has been shown to be associated with Aβ deposition and progression of mild cognitive impairment to Alzheimer’s disease.

Brain performance tests were found to correlate with levels of both proteins, with p-tau217 showing the strongest association.

Capillary p-tau217 was significantly higher in people with dementia compared to those without and was significantly associated with cognitive performance and function.

A combination of an 85% specificity threshold for capillary p-tau217 85% and episodic memory performance one standard deviation (SD) below benchmarked norms identified 9% of participants who were at potentially high risk, and who also showed significantly higher impairment in cognition and function.

Importantly, this threshold for impairment of episodic memory indicated a much milder level of impairment than the 1.5 SD change required to identify people with mild cognitive impairment, revealing its potential ability to spot signs at a preclinical stage.

Unexpectedly, even though ptau217 and GFAP both identified individuals with cognitive impairment, there was only a modest overlap in individuals who were positive for both GFAP and p-tau217, with GFAP identifying a different group of at-risk individuals. GFAP biomarker appeared to be associated with vascular risk, unlike p-tau217.

Researcher Clive Ballard, MD, PhD, also at Exeter, said: “Our approach of combining our robust cognitive testing with measuring proteins via a postal blood test could provide a straightforward, efficient and cost-effective method to reach large numbers of people in the community who would not otherwise be prioritized for the next steps of diagnosis or support and to optimize the clinical pathway to enable early detection of those at highest risk.”

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Biomarkers of ASD/ADHD and Factors Affecting Anxiety and Depression in Children and Young Adults

Conditions: ADHD – Attention Deficit Disorder With Hyperactivity; Autism Spectrum Disorder (ASD); Developmental Coordination Disorder (DCD)

Sponsors: University of Exeter; University of Southampton; University of Dublin, Trinity College; Carol Davila University of Medicine and Pharmacy; Jimma University; FUNDACION PARA LA INVESTIGACION HOSPITAL CLINICO SAN CARLOS; The International Centre for Diarrhoeal Disease Research, Bangladesh; University of Bari Aldo Moro

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