2021年12月1日 · Studies have shown that LGI1 plays an essential role during development, but also in neuronal excitability through an action on voltage-gated potassium Kv1.1 channels, and in synaptic transmission by regulating the surface expression of α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptors (AMPA-R).

2018年11月1日 · Human autoantibodies against LGI1 associate with a form of autoimmune limbic encephalitis characterized by severe but treatable memory impairment and frequent faciobrachial dystonic seizures. Although there is evidence that this disease is immune-mediated, the underlying LGI1 antibody-mediated mechanisms are unknown.

2006年3月2日 · Here, we show that Kv1.1-containing channels are complexed with Lgi1, the functionally unassigned product of the leucine-rich glioma inactivated gene 1 (LGI1), which is causative for an autosomal dominant form of lateral temporal lobe epilepsy (ADLTE).

2006年3月2日 · The voltage-gated potassium (Kv) channel subunit Kv1.1 is a major constituent of presynaptic A-type channels that modulate synaptic transmission in CNS neurons. Here, we show that Kv1.1-containing channels are complexed with Lgi1, the functionally unassigned product of the leucine-rich glioma inacti …

Functional studies showed that Lgi1 effectively and specifically prevented rapid voltage-gated potassium channel inactivation mediated by the Kv-beta-1 subunit (KCNAB1; 601141). In other words, Lgi1 effectively upregulates Kcna1 channels.

2024年6月15日 · In animal models of LGI1-dependent autosomal dominant lateral temporal lobe epilepsy, Kv 1 channels are downregulated, suggesting their crucial involvement in epileptogenesis.

2018年10月20日 · Petit-Pedrol et al. demonstrate in a mouse model of cerebroventricular antibody transfer that anti-LGI1 antibodies cause impairment of memory and plasticity, neuronal hyperexcitability, and decreased levels of Kv1.1 potassium channels and AMPA receptors. Keywords: limbic encephalitis, LGI1, AMPA receptors, Kv1.1, synaptic hyperexcitability.

In this study we analyse by immunofluorescence the modifications in neuronal Kv 1.1 and Kv 1.2 distribution throughout the hippocampal formation of LGI1 knock-out mice. We show that Kv 1 downregulation is not restricted to the axonal compartment, but also takes place in the somatodendritic region and is accompanied by a drastic decrease in Kv 2 ...

2024年5月28日 · 研究人员通过免疫荧光技术分析了Lgi1基因缺失小鼠海马区神经元Kv_1.1和Kv_1.2的分布变化。 他们发现Kv₁通道的下调不仅局限于轴突区域，还发生在体突区域，并伴随着Kv₂表达水平的显著下降。

2023年7月28日 · In animal models of LGI1-dependent autosomal dominant lateral temporal lobe epilepsy (ADTLE), Kv1 channels are downregulated, suggesting their crucial involvement in epileptogenesis. The molecular basis of Kv1 channel-downregulation in LGI1 knock-out mice has not been elucidated and how the absence of this extracellular protein induces an ...