Skip to content
Review Open access

Rare Variants in Purinergic P2X Receptor Genes (P2RX4, P2RX5, P2RX7) in Individuals With Autism Spectrum Disorder: An Exploratory Study

Jan 2026 · Human Mutation · Vol 2026 · 0 citations · 24 references
Medicine

TL;DR

Preliminary, exploratory observations suggesting that rare P2RX variants may represent biologically plausible candidates relevant to ASD‐related pathways are provided; however, no conclusions regarding disease association or causality can be drawn.

Abstract

Background Purinergic P2X receptors (P2RX) play key roles in neuroinflammatory processes through ATP‐gated ion channel signaling. Dysregulation of P2RX receptor activity has been implicated in maternal immune activation, mitochondrial dysfunction, oxidative stress, and synaptic abnormalities associated with autism spectrum disorder (ASD). Despite increasing mechanistic evidence, the contribution of P2RX gene variants to ASD remains poorly understood. Methods We retrospectively evaluated whole‐exome sequencing (WES) data from individuals diagnosed with ASD according to DSM‐5 criteria. Variants in the P2RX1–P2RX7 genes were identified. Clinical, genetic, and in silico data were reviewed, and variants were classified according to ACMG/AMP guidelines. Results Eleven individuals were identified with heterozygous P2RX variants, most frequently in P2RX7 (54.5%), followed by P2RX5 (27.3%) and P2RX4 (18.2%). Core clinical features across all genes included ASD, intellectual disability (ID), and epilepsy. P2RX4 variants included an in‐frame deletion (p.Ile322del) and a truncating variant (p.Tyr366LeufsTer7), both observed in individuals with ASD and ID. P2RX5 variants comprised rare missense substitutions with variable in silico pathogenicity. Several P2RX7 variants, most notably the canonical splice‐site variant c.614 + 1G > A fulfilling ACMG/AMP PVS1 and PM2 criteria, were predicted to alter receptor function. Overall, in silico analyses indicated moderate to high predicted functional impact; however, most variants lacked segregation and functional validation data. Conclusion Our findings provide preliminary, exploratory observations suggesting that rare P2RX variants may represent biologically plausible candidates relevant to ASD‐related pathways; however, no conclusions regarding disease association or causality can be drawn. These findings should be considered hypothesis‐generating and require validation through larger controlled genetic studies and functional investigations.

Read PDF

Similar papers

Open access Jul 2026

Expanded molecular pathomechanisms of GlyR α2 subunit variants in neurodevelopmental disorders and epileptic encephalopathy

These findings expand the clinical and mechanistic spectrum of GlyR α2 variants, identify a key determinant of picrotoxin binding, and highlight unresolved roles of intracellular protein-protein interaction motifs in GlyR α2 function.

Sean D. Fraser, Anna-Lena Wiessler, W. Choi et al. · 0 citations
Open access Aug 2026

Insights from GWAS, TWAS, and DEG analysis: CDKN2B-AS1, MAP3K4, and P2RX2-encoded P2X2 receptor identified as potential therapeutic targets for ischemic heart disease in Russian adults and long-living individuals

Background The purpose of this study was to investigate genetic risk factors associated with ischemic heart disease (IHD) in Russian adults, to develop prognostic PRS models for IHD risk stratification, and to gain a better understanding of molecular mechanisms underlying IHD through a transcriptome analysis. Materials...

Veronika Daniel, Наталья Георгиевна Романова, A. Mamchur et al. · 0 citations
Review Aug 2026

Identifying the Potential Role of Missense KIRREL3 Variants in Neurodevelopmental Phenotypes: A Case Series.

Neurodevelopmental disorders encompass a large group of conditions, many of which can be explained by genetic variants. KIRREL3 has previously been associated with neurodevelopmental disorders and is expressed in the developing human basal ganglia and amygdala. Through GeneMatcher, which allows clinicians, families, an...

Priyanka R Narayan, Sabah Sabir, Divya Jayvas et al. · 0 citations
Review Open access Aug 2026

PPP1R21-Related neurodevelopmental disorder: Phenotypic delineation, variant spectrum, and pathophysiological mechanisms

PPP1R21-related neurodevelopmental disorder (PPP1R21-NDD) is an ultra-rare autosomal-recessive encephalopathy caused by dysfunction of the Five-subunit Endosomal Rab5 and RNA/ribosome intermediarY (FERRY) complex. To delineate the clinical, neuroimaging, and molecular spectrum of PPP1R21-NDD and outline diagnostic and...

F. Comisi, G. Di Pasquale, A. Comisi et al. · 0 citations
Open access Aug 2026

Heterozygous Variants in LRP1 Cause a Neurodevelopmental Disorder with Congenital Heart Defects

The findings suggest that LRP1 haploinsufficiency is associated with a syndromic NDD, and Phenotypic differences in cardiac and neurologic involvement between participants with pLOF and missense variants suggest the possibility of alternate disease mechanisms.

Alyssa L. Rippert, G. Arnadottir, Laura Bedinger et al. · 0 citations
Open access Sep 2026

From genes to pathways: genetic convergence in early-onset Parkinsons disease in India

The first pathway resolved South Asian genetic profile and a framework for comparative studies across populations are provided, with different contributions from P/LP-associated and VUS associated gene sets.

R. Menon, A. I. Khan, D. Elangovan et al. · 0 citations

We use cookies to run the site and, with your consent, for analytics and to show ads. See our Cookie Policy.