Neuroserpin encoded by the SERPINI1 gene in humans is a member of the serine proteinase inhibitor (serpin) family. It reacts preferentially with tissue-type plasminogen activator (tPA). Neuroserpin is expressed mainly in the central nervous system (CNS) in response to neuronal depolarization and plays an important role in the development of synaptic plasticity. Mutations in human neuroserpin result in a form of autosomal dominant inherited dementia which is characterized by the presence of intraneuronal inclusion bodies and is known as Familial Encephalopathy with Neuroserpin Inclusion Bodies.
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