Observational studyFrontiers in immunology2026
Immunological characterization of neuronal intranuclear inclusion disease with kidney injury: an exploratory analysis in a multi-center cohort.
Observational study in Frontiers in immunology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
2 citing papers in PubMed.
- Editorial: Unraveling protein misfolding and innate immunity in neurodegenerative diseases.Frontiers in immunology · 2026Article
- High burden of kidney involvement in neuronal intranuclear inclusion disease.Frontiers in neurology · 2026Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
18 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Background: Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disorder caused by GGC repeat expansions in NOTCH2NLC, leading to uN2CpolyG protein deposition. Although immune-mediated renal lesions have been described in NIID, the systemic immunoinflammatory profile associated with kidney injury and its relationship with genetic burden remain undefined. This study investigated peripheral immune alterations in NIID-related nephropathy and their correlation with repeat expansion size. Methods: This multicenter retrospective study enrolled 150 genetically and pathologically confirmed NIID patients from nine tertiary hospitals (2019-2024). Using KDIGO criteria, patients were stratified into NIID with kidney injury (NIID-KD, n=100) and NIID with no kidney injury (NIID-ND, n=50) groups. Peripheral inflammatory markers were assessed in 110 patients, and 12 T-cell-related cytokines were measured in a subset of 35 patients. Multivariable analyses adjusting for disease duration were performed. GGC repeat numbers were quantified in 48 patients. Results: After adjustment for disease duration, the NIID-KD group maintained significantly higher white blood cell counts, neutrophil counts, monocyte counts, and neutrophil-to-lymphocyte ratio compared to NIID-ND (all Conclusion: NIID-associated kidney injury is characterized by a distinct immunoinflammatory signature with sustained neutrophilic and monocytic activation and IL-6 upregulation, suggesting involvement of innate immunity and IL-6-mediated inflammation in renal pathology. The absence of direct correlation between repeat expansion size and kidney involvement indicates that while genetic mutation confers disease susceptibility, acquired inflammatory mechanisms critically determine renal phenotype. These findings provide clinical evidence linking proteinopathy to innate immune activation in NIID.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.