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Items: 1 to 20 of 21

1.

Functional and dysfunctional conformers of human neuroserpin characterized by optical spectroscopies and Molecular Dynamics.

Noto R, Santangelo MG, Levantino M, Cupane A, Mangione MR, Parisi D, Ricagno S, Bolognesi M, Manno M, Martorana V.

Biochim Biophys Acta. 2015 Feb;1854(2):110-7. doi: 10.1016/j.bbapap.2014.10.002. Epub 2014 Nov 6.

2.

Lectin OS-9 delivers mutant neuroserpin to endoplasmic reticulum associated degradation in familial encephalopathy with neuroserpin inclusion bodies.

Schipanski A, Oberhauser F, Neumann M, Lange S, Szalay B, Krasemann S, van Leeuwen FW, Galliciotti G, Glatzel M.

Neurobiol Aging. 2014 Oct;35(10):2394-403. doi: 10.1016/j.neurobiolaging.2014.04.002. Epub 2014 Apr 8.

PMID:
24795221
3.

Sterol metabolism regulates neuroserpin polymer degradation in the absence of the unfolded protein response in the dementia FENIB.

Roussel BD, Newton TM, Malzer E, Simecek N, Haq I, Thomas SE, Burr ML, Lehner PJ, Crowther DC, Marciniak SJ, Lomas DA.

Hum Mol Genet. 2013 Nov 15;22(22):4616-26. doi: 10.1093/hmg/ddt310. Epub 2013 Jun 28.

4.

Sorafenib in elderly patients with advanced hepatocellular carcinoma: a case series.

Montella L, Addeo R, Cennamo G, Vincenzi B, Palmieri R, Sperlongano P, Sperlongano R, Iodice P, Russo P, Del Prete S.

Oncology. 2013;84(5):265-72. doi: 10.1159/000345558. Epub 2013 Feb 20.

PMID:
23428832
5.

A novel interaction between aging and ER overload in a protein conformational dementia.

Schipanski A, Lange S, Segref A, Gutschmidt A, Lomas DA, Miranda E, Schweizer M, Hoppe T, Glatzel M.

Genetics. 2013 Mar;193(3):865-76. doi: 10.1534/genetics.112.149088. Epub 2013 Jan 18.

6.

Endoplasmic reticulum dysfunction in neurological disease.

Roussel BD, Kruppa AJ, Miranda E, Crowther DC, Lomas DA, Marciniak SJ.

Lancet Neurol. 2013 Jan;12(1):105-18. doi: 10.1016/S1474-4422(12)70238-7. Review.

PMID:
23237905
7.

The tempered polymerization of human neuroserpin.

Noto R, Santangelo MG, Ricagno S, Mangione MR, Levantino M, Pezzullo M, Martorana V, Cupane A, Bolognesi M, Manno M.

PLoS One. 2012;7(3):e32444. doi: 10.1371/journal.pone.0032444. Epub 2012 Mar 6.

8.

The serpinopathies studying serpin polymerization in vivo.

Irving JA, Ekeowa UI, Belorgey D, Haq I, Gooptu B, Miranda E, Pérez J, Roussel BD, Ordóñez A, Dalton LE, Thomas SE, Marciniak SJ, Parfrey H, Chilvers ER, Teckman JH, Alam S, Mahadeva R, Rashid ST, Vallier L, Lomas DA.

Methods Enzymol. 2011;501:421-66. doi: 10.1016/B978-0-12-385950-1.00018-3.

PMID:
22078544
9.

Unravelling the twists and turns of the serpinopathies.

Roussel BD, Irving JA, Ekeowa UI, Belorgey D, Haq I, Ordóñez A, Kruppa AJ, Duvoix A, Rashid ST, Crowther DC, Marciniak SJ, Lomas DA.

FEBS J. 2011 Oct;278(20):3859-67. doi: 10.1111/j.1742-4658.2011.08201.x. Epub 2011 Jun 20. Review.

10.

The endoplasmic reticulum (ER)-associated degradation system regulates aggregation and degradation of mutant neuroserpin.

Ying Z, Wang H, Fan H, Wang G.

J Biol Chem. 2011 Jun 10;286(23):20835-44. doi: 10.1074/jbc.M110.200808. Epub 2011 Apr 20.

11.

Characterisation of serpin polymers in vitro and in vivo.

Belorgey D, Irving JA, Ekeowa UI, Freeke J, Roussel BD, Miranda E, Pérez J, Robinson CV, Marciniak SJ, Crowther DC, Michel CH, Lomas DA.

Methods. 2011 Mar;53(3):255-66. doi: 10.1016/j.ymeth.2010.11.008. Epub 2010 Nov 27. Review.

PMID:
21115126
12.

Neuroserpin polymers activate NF-kappaB by a calcium signaling pathway that is independent of the unfolded protein response.

Davies MJ, Miranda E, Roussel BD, Kaufman RJ, Marciniak SJ, Lomas DA.

J Biol Chem. 2009 Jul 3;284(27):18202-9. doi: 10.1074/jbc.M109.010744. Epub 2009 May 7.

13.

The 2.1-A crystal structure of native neuroserpin reveals unique structural elements that contribute to conformational instability.

Takehara S, Onda M, Zhang J, Nishiyama M, Yang X, Mikami B, Lomas DA.

J Mol Biol. 2009 Apr 24;388(1):11-20. doi: 10.1016/j.jmb.2009.03.007. Epub 2009 Mar 10.

PMID:
19285087
14.

Human neuroserpin: structure and time-dependent inhibition.

Ricagno S, Caccia S, Sorrentino G, Antonini G, Bolognesi M.

J Mol Biol. 2009 Apr 24;388(1):109-21. doi: 10.1016/j.jmb.2009.02.056. Epub 2009 Mar 2.

PMID:
19265707
15.

Mutation-, aging-, and gene dosage-dependent accumulation of neuroserpin (G392E) in endoplasmic reticula and lysosomes of neurons in transgenic mice.

Takasawa A, Kato I, Takasawa K, Ishii Y, Yoshida T, Shehata MH, Kawaguchi H, Mohafez OM, Sasahara M, Hiraga K.

J Biol Chem. 2008 Dec 19;283(51):35606-13. doi: 10.1074/jbc.M804125200. Epub 2008 Oct 21.

16.

The intracellular accumulation of polymeric neuroserpin explains the severity of the dementia FENIB.

Miranda E, MacLeod I, Davies MJ, Pérez J, Römisch K, Crowther DC, Lomas DA.

Hum Mol Genet. 2008 Jun 1;17(11):1527-39. doi: 10.1093/hmg/ddn041. Epub 2008 Feb 11.

17.

A structural basis for loop C-sheet polymerization in serpins.

Zhang Q, Law RH, Bottomley SP, Whisstock JC, Buckle AM.

J Mol Biol. 2008 Mar 7;376(5):1348-59. doi: 10.1016/j.jmb.2007.12.050. Epub 2008 Jan 3.

PMID:
18234218
18.

Clinical and neuropathologic study of a French family with a mutation in the neuroserpin gene.

Gourfinkel-An I, Duyckaerts C, Camuzat A, Meyrignac C, Sonderegger P, Baulac M, Brice A.

Neurology. 2007 Jul 3;69(1):79-83.

PMID:
17606885
19.

Accumulation of mutant neuroserpin precedes development of clinical symptoms in familial encephalopathy with neuroserpin inclusion bodies.

Galliciotti G, Glatzel M, Kinter J, Kozlov SV, Cinelli P, Rülicke T, Sonderegger P.

Am J Pathol. 2007 Apr;170(4):1305-13.

20.

Protein misfolding and the serpinopathies.

Belorgey D, Hägglöf P, Karlsson-Li S, Lomas DA.

Prion. 2007 Jan-Mar;1(1):15-20. Epub 2007 Jan 6. Review.

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