neuroserpinrelated

Neuroserpin-related refers to aspects of biology, disease, and research connected to neuroserpin, a neuron-specific serine protease inhibitor encoded by the SERPINI1 gene and expressed predominantly in the brain. Neuroserpin inhibits tissue plasminogen activator (tPA) and modulates extracellular proteolysis, contributing to regulation of synaptic plasticity, neuronal survival, and responses to injury. Through its anti-proteolytic activity, neuroserpin influences remodeling of neural networks and protective pathways after neuronal stress.

Genetic and molecular underpinnings center on SERPINI1. The most well-described neuroserpin-related disorder is familial encephalopathy with neuroserpin inclusion bodies (FENIB), an autosomal dominant neurodegenerative disease caused by SERPINI1 mutations that promote misfolding and polymerization of neuroserpin. Polymeric neuroserpin accumulates in neurons’ endoplasmic reticulum, forming inclusion bodies that trigger ER stress and progressive cognitive and motor impairment, often with onset in adulthood.

Beyond FENIB, alterations in neuroserpin activity and its interaction with tPA have been investigated in stroke and other neurodegenerative contexts. Experimental models suggest that neuroserpin can reduce ischemic injury by limiting excessive proteolysis, while dysregulation may contribute to pathology by promoting inflammation or synaptic dysfunction. Therapeutic approaches have explored boosting neuroserpin activity or delivering mimetics, though clinical translation remains under study.

In summary, neuroserpin-related literature encompasses basic biology of neuronal tPA inhibition, SERPINI1-linked diseases such as FENIB, and exploration of neuroserpin’s potential as a therapeutic target in brain injury and neurodegeneration.