Serpin peptidase inhibitor, clade I (neuroserpin), member 1 (SERPINI1)

Serpin peptidase inhibitor, clade I (neuroserpin), member 1 (SERPINI1), commonly known as neuroserpin, is a secreted serine protease inhibitor of the serpin superfamily predominantly expressed in the nervous system[1][2][3]. Neuroserpin regulates neuronal growth and synaptic plasticity by inhibiting tissue-type plasminogen activator (tPA), thus influencing processes like neuronal migration, synapse development, and protection against excitotoxic injury[1][2]. Mutations of SERPINI1 lead to familial encephalopathy with neuroserpin inclusion bodies (FENIB), a neurodegenerative disorder resulting from aggregated, misfolded neuroserpin in neurons, causing dementia, epilepsy, and neurodegeneration[1][2]. Neuroserpin is additionally implicated in Alzheimer’s disease pathology and may act as a protective factor in acute stroke, with roles expanding into cancer cell biology and immune regulation[2]. No direct agonists or antagonists are approved for clinical use; research focuses on its impact in neurological and systemic diseases, particularly those involving neurodegeneration and cerebrovascular injury[2].

Other names

NeuroserpinPI12PI-12Peptidase inhibitor 12Serpin I1HNS-S1HNS-S2Serpin family I member 1Serpin peptidase inhibitor clade I member 1Protease inhibitor 12 (neuroserpin)Serine (or cysteine) proteinase inhibitor, clade I (neuroserpin), member 1

Inhibition of tissue-type plasminogen activator (tPA) activity in the nervous system[1][2]; Modulation of proteolytic balance impacting extracellular matrix remodeling, synaptic development, and amyloid beta clearance[2]; Mitigation of neuronal apoptosis and excitotoxicity after cerebral ischemia (by exogenous neuroserpin)[2]