B6-hSNCA (3'UTR) Mouse

Parkinson's disease (PD) is a neurodegenerative disease with a high prevalence mainly in the middle-aged and elderly population. It is the second most common neurodegenerative disease after Alzheimer's disease (AD). The main clinical symptoms include resting tremors, limb stiffness, bradykinesia, loss of voluntary movement, etc. The typical pathological process of PD is the formation of Lewy bodies (LB) in the central nervous system (CNS), which results in the gradual death and loss of dopaminergic neurons, leading to the disease ^[1-2]. The main components of Lewy bodies are insoluble aggregates of abnormal α-synuclein (α-syn), and the SNCA gene, which encodes α-synuclein, is one of the key causative genes in Parkinson's disease. Mutations in this gene cause overexpression of α-syn, leading to Lewy bodies' formation, ultimately leading to PD ^[3]. In addition, SNCA mutations are also associated with diseases such as dementia with Lewy bodies (DLB) and multiple system atrophy (MSA).

The B6-hSNCA (3'UTR) mouse is a humanized model constructed by replacing the mouse Snca gene in situ with the human SNCA gene, in which the sequences from ATG start codon to downstream of 3'UTR of the endogenous mouse Snca gene are replaced with the sequences from ATG start codon to downstream of 3'UTR of the human SNCA gene. The homozygous B6-hSNCA (3'UTR) mice are viable and fertile and can be used for studies on Parkinson's disease (PD), Dementia with Lewy Bodies (DLB) and Multiple System Atrophy (MSA), and pathogenesis of neurodegenerative diseases, as well as for SNCA-targeted drug development.