The DOI matches its Crossref record; 75 of the 75 references it cites were found in a registry or an index.
Alpha1-Antitrypsin Deficiency — A Model for Conformational Diseases
Abstract
Alpha1-antitrypsin is a member of a family of protease inhibitors known as the serpins. Mutations in these molecules can lead to disease, not only because the biologic activity of the protease in tissue is increased, but also because the mutations result in misfolded (i.e., conformationally abnormal) protease molecules that accumulate in tissue. This review article summarizes the action of these protease inhibitors and how mutations lead to their accumulation in particular neurodegenerative disorders such as prion encephalopathies and Alzheimer's disease.
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Cites
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Aβ peptide vaccination prevents memory loss in an animal model of Alzheimer's disease
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The Electrophoretic α; 1 -Globulin Pattern of Serum in α; 1 -Antitrypsin Deficiency
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Translating cell biology into therapeutic advances in Alzheimer's disease
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Structure of a serpin–protease complex shows inhibition by deformation
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Self-citationThe mechanism of Z α1-antitrypsin accumulation in the liver
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Self-citationThe alternative conformations of amyloidogenic proteins and their multi-step assembly pathways
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Metabolic Regulation of Brain Aβ by Neprilysin
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ACG clinical guideline: evaluation of abnormal liver chemistries
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Mediators of Chronic Obstructive Pulmonary Disease
Barnes, P. J. (2004) · Pharmacological Reviews · doi:10.1124/pr.56.4.2 · Cited by 710
Grupe, A., Abraham, R., Li, Y., Rowland, C., Hollingworth, P., Morgan, A., Jehu, L., Segurado, R., Stone, D., Schadt, E., et al. (2007) · Human Molecular Genetics · doi:10.1093/hmg/ddm031 · Cited by 289
Increased production of sonic hedgehog by ballooned hepatocytes
Rangwala, F., Guy, C. D., Lu, J., Suzuki, A., Burchette, J. L., Abdelmalek, M. F., Chen, W., & Diehl, A. M. (2011) · The Journal of Pathology · doi:10.1002/path.2888 · Cited by 201
O’Brien, M. E., Murray, G., Gogoi, D., Yusuf, A., McCarthy, C., Wormald, M. R., Casey, M., Gabillard-Lefort, C., McElvaney, N. G., & Reeves, E. P. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms23052441 · Cited by 91
Metadata
- Authors
- Carrell, R. W., & Lomas, D. A.
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 2002-01-03
- Volume
- 346
- Issue
- 1
- Pages
- 45-53
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W2066777346
- Cited by
- 448
- References
- 75
- FWCI
- 13.39
- Type
- journal article
- Language
- en
- Open access
- closed
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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
