huSTMN2 Mouse
Product Name
huSTMN2 Mouse
Product ID
C001959
Strain Name
C57BL/6JCya-Stmn2tm1(hSTMN2)/Cya
Backgroud
C57BL/6JCya
Status
When using this mouse strain in a publication, please cite “huSTMN2 Mouse (Catalog C001959) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
Gene Name
STMN2
Gene Alias
SCG10, SCGN10
NCBI ID
Chromosome
Chr 8 (Human)
MGI ID
Datasheet
Strain Description
The STMN2 gene encodes the stathmin-2 protein, a microtubule-associated protein and member of the stathmin family. It plays a critical role in neuronal development, axonal growth, and regeneration by regulating microtubule dynamic stability. The STMN2 gene is predominantly expressed in the nervous system, with the highest levels in central and peripheral neurons, serving as a key molecule for maintaining axonal integrity and regenerative capacity. Additionally, its expression is elevated in developing neurons and is also present at moderate levels in adult brain tissues and the adrenal gland. Studies have shown that loss of TDP-43 function leads to cryptic splicing or premature polyadenylation of STMN2 mRNA, resulting in a significant reduction in functional STMN2 protein levels. This, in turn, causes neuromuscular junction denervation and axonal degeneration, which can be effectively reversed by exogenous supplementation of STMN2 or its post-translational stabilization [1-2]. In clinical research, the reduction of STMN2 due to TDP-43 pathology is a common feature in the majority of patients with amyotrophic lateral sclerosis (ALS) and can serve as a disease biomarker and potential therapeutic target [3]. Furthermore, STMN2 regulates α-synuclein (α-syn) aggregation and dopaminergic axonal integrity. It is significantly downregulated in the brain tissue of patients with Parkinson’s disease (PD), leading to dopaminergic neuron degeneration, elevated phosphorylated α-syn, and motor deficits. It has been identified as a key regulator functionally connected to known PD risk genes [4-5]. Studies have shown that humanized mouse models can precisely recapitulate STMN2-related pathological mechanisms. By delivering shRNA via adeno-associated virus (AAV), these models enable central nervous system-specific regulation, providing a robust tool platform for investigating the mechanisms of Parkinson’s disease (PD) [6].
The huSTMN2 mouse is a humanized model constructed using gene editing technology. The sequences from upstream of the exon 1 to downstream of the exon 5 of the mouse Stmn2 were replaced with the sequences from upstream of the exon 1 to downstream of the exon 5 of the human STMN2. The huSTMN2 mice can be used to investigate the pathogenesis and progression of neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS) and Parkinson’s disease (PD), facilitating the research and development of STMN2-targeted drugs and gene therapy strategies, as well as preclinical pharmacological and efficacy evaluations.
Reference
Melamed Z, López-Erauskin J, Baughn MW, et al. Premature polyadenylation-mediated loss of stathmin-2 is a hallmark of TDP-43-dependent neurodegeneration. Nat Neurosci. 2019;22(2):180-190.
Guerra San Juan I, Nash LA, Smith KS, et al. Loss of mouse Stmn2 function causes motor neuropathy. Neuron. 2022;110(10):1671-1688.e6.
Klim JR, Williams LA, Limone F, et al. ALS-implicated protein TDP-43 sustains levels of STMN2, a mediator of motor neuron growth and repair. Nat Neurosci. 2019;22(2):167-179.
Wang Q, Zhang Y, Wang M, et al. The landscape of multiscale transcriptomic networks and key regulators in Parkinson's disease. Nat Commun. 2019;10(1):5234.
San Juan IG, Nash LA, Smith KS, et al. Loss of mouse Stmn2 function causes motor neuropathy. Neuron. 2022;110(10):1671-1688.e6.
Pickles, Sarah R., Gaiani, Luca., Lin, Lilian., Jansen-West, Karen., and Tong, Jimei.. "Basic Science and Pathogenesis." Alzheimer's & dementia : the journal of the Alzheimer's Association.
Strain Strategy
The sequences from upstream of the exon 1 to downstream of the exon 5 of the mouse Stmn2 were replaced with the sequences from upstream of the exon 1 to downstream of the exon 5 of the human STMN2.
Figure 1. Gene editing strategy of huSTMN2 mice.
Application Area
The screening, development, and preclinical evaluation of STMN2-targeted drugs;
Research on the pathogenesis and therapies of neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS) and Parkinson's disease (PD);
Preclinical efficacy evaluation of STMN2 gene therapy strategies.
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