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 ?
Association between conformational mutations in neuroserpin and onset and severity of dementia.
No quantitative assay for neuroserpin was available at the time of these experiments.
2 Mouse IgG1K Neuroserpin aa 145-154 DNA and GST- neuroserpin protein boost for all 4421 5B5.