High-throughput behavioural phenotyping of 25 C. elegans disease models including patient-specific mutations

对包括患者特异性突变在内的25种秀丽隐杆线虫疾病模型进行高通量行为表型分析

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Abstract

BACKGROUND: Genetic diagnosis is fast and cheap, challenging our capacity to evaluate the functional impact of novel disease-causing variants or identify potential therapeutics. Model organisms including C. elegans present the possibility of systematically modelling genetic diseases, yet robust, high-throughput methods have been lacking. RESULTS: Here we show that automated multi-dimensional behaviour tracking can detect phenotypes in 25 new C. elegans disease models spanning homozygous loss-of-function alleles and patient-specific single-amino-acid substitutions. We find that homozygous loss-of-function (LoF) mutants across diverse genetic pathways (including BORC, FLCN, and FNIP-2) exhibit strong, readily detectable abnormalities in posture, locomotion, and stimulus responses compared to wild-type animals. An smc-3 mutant strain-modelled by introducing a patient-identified missense change-exhibited developmental anomalies and distinct behavioural profiles even though complete loss of SMC-3 is lethal. In contrast, patient-derived missense mutations in another essential gene, tnpo-2, did not show a strong phenotype initially but it could be "sensitized" chemically (e.g., with aldicarb), potentially facilitating future drug screens. CONCLUSIONS: Our findings show that scalable behavioural phenotyping can capture a wide range of mutant effects-from strong to subtle-in patient-avatar worm lines. We anticipate that this standardized approach will enable systematic drug repurposing for rare genetic disorders as new disease variants are discovered.

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