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Items: 1 to 20 of 113

1.

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.

2.

The aggregation-prone intracellular serpin SRP-2 fails to transit the ER in Caenorhabditis elegans.

Silverman RM, Cummings EE, O'Reilly LP, Miedel MT, Silverman GA, Luke CJ, Perlmutter DH, Pak SC.

Genetics. 2015 May;200(1):207-19. doi: 10.1534/genetics.115.176180. Epub 2015 Mar 18.

3.
4.

Mutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulum.

Miranda E, Römisch K, Lomas DA.

J Biol Chem. 2004 Jul 2;279(27):28283-91. Epub 2004 Apr 16.

5.

Latent S49P neuroserpin forms polymers in the dementia familial encephalopathy with neuroserpin inclusion bodies.

Onda M, Belorgey D, Sharp LK, Lomas DA.

J Biol Chem. 2005 Apr 8;280(14):13735-41. Epub 2005 Jan 21.

6.

A rat model of human FENIB (familial encephalopathy with neuroserpin inclusion bodies).

Takano K, Kitao Y, Inagi R, Momoi T, Matsuyama T, Miyata T, Yoneda Y, Iso H, Stern DM, Hori O, Ogawa S.

Biochem Biophys Res Commun. 2006 Aug 4;346(3):1040-7. Epub 2006 Jun 12.

PMID:
16782060
7.

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
8.

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.

9.

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
10.

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.

11.

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.

12.

Limited Unfolded Protein Response and Inflammation in Neuroserpinopathy.

López-González I, Pérez-Mediavilla A, Zamarbide M, Carmona M, Torrejón Escribano B, Glatzel M, Galliciotti G, Ferrer I.

J Neuropathol Exp Neurol. 2016 Feb;75(2):121-33.

PMID:
26733586
13.

Interactions between N-linked glycosylation and polymerisation of neuroserpin within the endoplasmic reticulum.

Moriconi C, Ordoñez A, Lupo G, Gooptu B, Irving JA, Noto R, Martorana V, Manno M, Timpano V, Guadagno NA, Dalton L, Marciniak SJ, Lomas DA, Miranda E.

FEBS J. 2015 Dec;282(23):4565-79. doi: 10.1111/febs.13517. Epub 2015 Oct 3.

14.

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.

15.
16.

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.

17.

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.

18.

Familial dementia caused by polymerization of mutant neuroserpin.

Davis RL, Shrimpton AE, Holohan PD, Bradshaw C, Feiglin D, Collins GH, Sonderegger P, Kinter J, Becker LM, Lacbawan F, Krasnewich D, Muenke M, Lawrence DA, Yerby MS, Shaw CM, Gooptu B, Elliott PR, Finch JT, Carrell RW, Lomas DA.

Nature. 1999 Sep 23;401(6751):376-9.

PMID:
10517635
19.
20.

Endoplasmic reticulum-associated degradation (ERAD) and autophagy cooperate to degrade polymerogenic mutant serpins.

Kroeger H, Miranda E, MacLeod I, Pérez J, Crowther DC, Marciniak SJ, Lomas DA.

J Biol Chem. 2009 Aug 21;284(34):22793-802. doi: 10.1074/jbc.M109.027102. Epub 2009 Jun 23.

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