Kaas Mathias, Chofflet Nicolas, Bicer Deniz, Skeldal Sune, Duan Jinjie, Feller Benjamin, Vilstrup Joachim, Groth Rosa, Sivagurunathan Suganya, Dashti Hesam, Pedersen Jan Skov, Werge Thomas, Børglum Anders D, Cimini Beth A, Jones Thouis R, Claussnitzer Melina, Madsen Peder, Takahashi Hideto, Demontis Ditte, Thirup Søren, Glerup Simon
Department of Biomedicine, Aarhus University, Aarhus, Denmark.
The Novo Nordisk Foundation Center for Genomic Mechanisms of Disease, Broad Institute of MIT and Harvard, Cambridge, USA.
bioRxiv. 2025 Feb 20:2025.02.16.638491. doi: 10.1101/2025.02.16.638491.
The leukocyte common antigen related receptor (LAR) is a member of the LAR receptor protein tyrosine phosphatase (RPTP) family of synaptic adhesion molecules that contribute to the proper alignment and specialization of synaptic connections in the mammalian brain. LAR-RPTP members have been genetically associated with neuropsychiatric disorders, but the molecular consequences of genetic perturbations of LAR remain unstudied. Using exome sequencing data from psychiatric patients and controls, we identify rare missense variants of LAR that render the extracellular domain (ECD) unstable and susceptible to proteolytic cleavage. Using recombinant and cellular systems, we describe three variants that cause disruption of the LAR:NGL-3 interaction, which results in loss of transcellular adhesion and synaptogenic effects. Furthermore, we show that overexpression of two of these variants elicit altered morphological phenotypes in an imaging-based morphological profiling assay compared to wild type LAR, suggesting that destabilization of the LAR ECD has broad effects on LAR function. In conclusion, our study identifies three rare, missense variants in LAR that could provide insights into LAR involvement with psychiatric pathobiology.
白细胞共同抗原相关受体(LAR)是LAR受体蛋白酪氨酸磷酸酶(RPTP)家族的成员,该家族属于突触粘附分子,有助于哺乳动物大脑中突触连接的正确排列和特化。LAR - RPTP成员在遗传上与神经精神疾病有关,但LAR基因扰动的分子后果仍未得到研究。利用来自精神疾病患者和对照的外显子组测序数据,我们鉴定出LAR的罕见错义变体,这些变体使细胞外结构域(ECD)不稳定并易受蛋白水解切割。使用重组和细胞系统,我们描述了三种导致LAR:NGL - 3相互作用破坏的变体,这导致跨细胞粘附和突触形成作用丧失。此外,我们表明,与野生型LAR相比,在基于成像的形态学分析中,其中两种变体的过表达引发了形态学表型的改变,这表明LAR ECD的不稳定对LAR功能有广泛影响。总之,我们的研究鉴定出LAR中的三种罕见错义变体,这可能为LAR参与精神疾病病理生物学提供见解。