Axonally released neuronal serine protease inhibitor found in cortical and hippocampal neurons; a familial form of presenile dementia has been linked to mutations of neuroserpins; mutant neuroserpins form polymers or inclusion bodies result in neuronal degeneration


A gene on chromosome 3q26.1 that encodes a member of the serpin superfamily of serine proteinase inhibitors that inhibits tissue-type plasminogen activator. It is secreted by axons in the brain and thought to play a role in regulating axonal growth and the development of synaptic plasticity or the reorganisation of synaptic connections. SERPINI1 may protect neurons from cell damage by tissue-type plasminogen activator.

Molecular pathology
SERPINI1 mutations cause familial encephalopathy with neuroserpin inclusion bodies (FENIB), an autosomal dominant form of familial encephalopathy and epilepsy.
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References in periodicals archive ?
Regulation of seizure spreading by neuroserpin and tissue-type plasminogen activator is plasminogen-independent.
Neuroserpin (NSP) is a serine protease inhibitor and member of the serpin family.
Local conformational flexibility provides a basis for facile polymer formation in human neuroserpin. Biophys J 20115;101:1758-65.
Association between neuroserpin and molecular markers of brain damage in patients with acute ischemic stroke.
Neuroserpin, a thrombolytic serine protease inhibitor (serpin), blocks transplant vasculopathy with associated modification of T-helper cell subsets.
* the up-regulation of the SERPINIi gene (serpin peptidase inhibitor, neuroserpin), which plays an important role in synapse development and regulates synaptic plasticity, and
Association between conformational mutations in neuroserpin and onset and severity of dementia.
Zygin, neuroserpin, and GAD67 were below the limit of detection of this method (<2.5 [micro]g/L), and the intensity of the VLP-1 band was between the 25 and 250 [micro]g/L VLP-1 standards.