Recombinant Human Neuroserpin (SERPINI1)

1. We present two pediatric cases of progressive myoclonic epilepsy with SERPINI1 pathogenic variants that lead to a severe presentation. PMID: 28631894
2. Data indicated that rs9853967 and rs11714980 polymorphisms in CCM3 and SERPINI1respectively could be associated with a protective role in cerebral cavernous malformations disease. PMID: 27737651
3. SERPINI1 is an important regulator of epithelial-mesenchymal transition in an orthotopic implantation model of colorectal cancer PMID: 26892864
4. The thermal and chemical stability along with the polymerisation propensity of both Wild Type and Glu289Ala NS were characterized. PMID: 26329378
5. This C-terminal lability is not required for neuroserpin polymerisation in the endoplasmic reticulum, but the additional glycan facilitates degradation of the mutant protein during proteasomal impairment. PMID: 26367528
6. the protective effect of neuroserpin maybe independent from its canonical interaction with a tissue-type plasminogen activator PMID: 26176694
7. Neuroserpin is expressed in naive effector memory and central memory CD4 and CD8 T cell subsets, and monocytes, B cells, and NK cells. T-cell activation caused its translocation to the immunologic synapse, secretion, and delayed downregulation. PMID: 25670787
8. Molecular Dynamics simulations suggest that Neuroserpin conformational stability and flexibility arise from a spatial distribution of intramolecular salt-bridges and hydrogen bonds. PMID: 25450507
9. Alzheimer's disease brain tissues with elevated neuroserpin protein also showed increased expression of THRbeta1 and HuD PMID: 24036060
10. our study did not provide any evidence for an association between genetic variation at the SERPINI1 locus and ischemic stroke PMID: 21487809
11. the origins of conformational lability PMID: 21961602
12. Neuroprotective properties of neuroserpin may be related to the inhibition of excitotoxicity, inflammation, as well as blood brain barrier disruption that occur after acute ischemic stroke. PMID: 21569344
13. Hrd1 and gp78 mediate mutant neuroserpin turnover through the ERAD pathway. PMID: 21507957
14. high serum neuroserpin levels before intravenous tPA and neuroserpin levels decrease at 24 h after ischaemic stroke, independently of tPA treatment, may have a role in good functional outcome PMID: 21174006
15. The latent and polymer hNS forms obtained at 45 degrees C and 85 degrees C differ in their chemical and thermal stabilities; furthermore, the human neuroserpin polymers also differ in size and morphology PMID: 21081089
16. investigated the refolding and polymerization pathways of wild-type neuroserpin and of the pathogenic mutants S49P and H338R PMID: 20691191
17. Mutant Neuroserpin (S49P) that causes familial encephalopathy with neuroserpin inclusion bodies is a poor proteinase inhibitor and readily forms polymers in vitro PMID: 11880376
18. The interactions between NSP and t-PA were distinct from those between plasmin and NSP, suggesting that the physiologic effect of t-PA-NSP interactions may be more complex than previously thought. PMID: 12228252
19. Neuroserpin has a role as a selective inhibitor of tissue-type plasminogen activator in the central nervous system [review] PMID: 14983220
20. neuroserpin mutants that cause dementia accumulate as polymers within the endoplasmic reticulum PMID: 15090543
21. tissue plasminogen activator and neuroserpin are widely expressed in the human central nervous system PMID: 15269833
22. reactive centre loop of neuroserpin Portland being partially inserted into beta-sheet A to adopt a conformation similar to an intermediate on the polymerization pathway PMID: 15291813
23. Data show that the S49P mutant of neuroserpin that causes the dementia familial encephalopathy with neuroserpin inclusion bodies (FENIB) forms a latent species in vitro and in vivo in addition to the formation of polymers. PMID: 15664988
24. neuroserpin interacts with Abeta(1-42) to form off-pathway non-toxic oligomers and so protects neurons in Alzheimer disease PMID: 16849336
25. intergenic region of the head-to-head PDCD10-SERPINI1 gene pair provides an interesting and informative example of a complex regulatory system PMID: 17212813
26. in a French family with the S52R mutation of the neuroserpin gene, progressive myoclonic epilepsy was associated with a frontal syndrome PMID: 17606885
27. This study provides the first evidence that neuroserpin is associated with early-onset ischemic stroke among Caucasian women. PMID: 17961231
28. conformational modification in the protein under oxidative stress PMID: 18051703
29. We report a neuroserpin mutation that causes electrical status epilepticus of slow-wave sleep. PMID: 18591508
30. Neuroserpin and tissue plasminogen activator are associated with amyloid-beta plaques in Alzheimer brain tissue. PMID: 19222708
31. Human neuroserpin: structure and time-dependent inhibition PMID: 19265707
32. Analyses restricted to glioblastoma (n = 254) yielded significant associations for the SELP, DEFB126/127, SERPINI1, and LY96 genetic regions. PMID: 19423540
33. intracellular neuroserpin polymers activate NF-kappaB by a pathway that is independent of the IRE1, ATF6, and PERK limbs of the canonical unfolded protein response but is dependent on intracellular calcium PMID: 19423713

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HGNC: 8943

OMIM: 602445

KEGG: hsa:5274

STRING: 9606.ENSP00000295777

UniGene: Hs.478153