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Article

Cellular Models for the Serpinopathies.

Methods in molecular biology (Clifton, N.J.) - 1 Jan 2018

Fra Annamaria, D'Acunto Emanuela, Laffranchi Mattia, Miranda Elena

Abstract excerpt

Our current knowledge about the cellular mechanisms underlying serpin-related disorders, the serpinopathies, is predominantly based on studies in cell culture models of disease, particularly for alpha-1 antitrypsin (AAT, SERPINA1) deficiency causing emphysema and the familial encephalopathy with neuroserpin (NS, SERPINI1) inclusion bodies (FENIB). FENIB, a neurodegenerative dementia, is caused by polymerization...

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Identifiers

PMID: 30194596
DOI: 10.1007/978-1-4939-8645-3_7

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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