Metrion Biosciences & The KCNC1 Foundation Collaborate on Development of New Ion Channel Modulators for Ultra-Rare Genetic Disorder
Metrion Biosciences Limited and The KCNC1 Foundation recently announced a collaboration to progress a hit identification research project for small molecule modulators of the potassium ion channel Kv3.1, targeting KCNC1-related disorders.
Variants of the KCNC1 gene impact Kv3.1 potassium channel function, resulting in neurodevelopmental disorders, which can include progressive myoclonic epilepsy (PME) and developmental epileptic encephalopathy (DEE). Symptoms of KCNC1 disorder include seizures during infancy, vision impairment, developmental delay, intellectual disability and many others. The collaboration is focused on the identification of small molecule modulators of the variant potassium ion channel, Kv3.1 as a potential therapeutic strategy for KCNC1-related disorders, identified as being the cause of KCNC1 related DEE. Metrion will develop cell lines for both wild type and variant Kv3.1, to establish a fluorescence-based screen using FLIPR Penta, a high-throughput cellular screening system. The high-throughput screen will then be used to identify novel inhibitors or activators of Kv3.1 mutant channels.
The KCNC1 Foundation was created by the parents of a child diagnosed with KCNC1-related DEE to support research aimed at accelerating the development of a cure, as well as to raise awareness to identify and connect patients and provide support for families.
“We are very excited to collaborate with Metrion on the possibility of identifying a potential therapeutic for children with KCNC1-related disorder. This is our first step of many to address the pathophysiology of the disorder, to give individuals suffering the opportunity to reach their full potential,” said Stephanie Telesca, Co-Founder of The KCNC1 Foundation. “We welcome any contributions toward The KCNC1 Foundation as we work hard to pave a path toward a cure.”
Dr. Andrew Southan, Metrion’s Chief Executive, added “I would like to thank The KCNC1 Foundation for choosing Metrion to progress this discovery research program. Our team looks forward to helping the Foundation accomplish its goal to deliver efficacious treatments to patients as quickly as possible.”
Metrion Biosciences is a specialist ion-channel contract research organization and drug discovery business. The company provides customers with access to a range of high-quality ion channel assays on a fee-for-service or collaboration basis. Metrion Biosciences’ ion channel expertise includes an industry leading panel of in vitro cardiac ion channel safety assays, translational native cell and phenotypic assays for neurological and cardiotoxicity testing, and a range of other ion channel screening services such as cell line development and optimization. Metrion Biosciences is able to provide tailored assay formats, data analysis and reporting solutions, effective project management and quality assured data packages. For more information, visit www.metrionbiosciences.com.
The KCNC1 Foundation is a Canadian parent-led organization that was established in 2022 to give individuals with KCNC1-related disorders a chance at a brighter future. We will accomplish this through supporting research to accelerate the path to a cure, identifying key partnerships to lead us through the drug development process, raising awareness to identify and connect patients affected by KCNC1-related disorders, and by providing support to their families. Since our inception, we have raised over $50,000 that has gone directly to funding research and connected 19 families worldwide. Donations can be made through gofundme (https://gofund.me/00b67750) or for those in the US, through our fiscal sponsor, The Rare Village: (https://give.rarevillage.org/give/411473/#!/donation/checkout). For more information, visit https://kcnc1.org.
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