mProX™ Human KCNC3 Stable Cell Line

Biosafety Level

Level 1

Activity

Yes

Quantity

10⁶ cells per vial

Applications

The voltage-gated potassium channel subunit Kv3.3, which is encoded by KCNC3, is responsible for driving the repolarization phase of action potentials and is highly expressed in cerebellar Purkinje cells. Spinocerebellar ataxia (SCA13), a disorder that can potentially include cognitive impairments and suggests both cerebellar and extracerebellar dysfunction, has been linked to mutations in the KCNC3. There are two missense mutations that have been identified; these mutations are likely to alter the properties of Purkinje cells' (and other neurons') output and disrupt the function of Kv3.3 channels in the repolarization of action potentials. For the three Kv3 genes (Kv3.1-Kv3.3) that are highly expressed in CNS neurons, knockout mice have been produced. The Kv3.1 and Kv3.3 double mutants exhibit severe ataxia, tremor, and myoclonus. The Kv3.1 and Kv3.3 knockout mice exhibit increased locomotor activity and sleep loss. The Kv3.3 knockout mice exhibit impaired gait and reduced motor performance associated with abnormal Purkinje cell discharges and abnormal olivocerebellar system properties. The customized KCNC3 stable cell line can be used in antibody discovery and development, potential drug candidate screening and signaling pathway researches.