Crowther DC - Search Results

1

Familial conformational diseases and dementias.

Crowther DC. Crowther DC. Hum Mutat. 2002 Jul;20(1):1-14. doi: 10.1002/humu.10100. Hum Mutat. 2002. PMID: 12112652 Review.

2

Mutant Neuroserpin (S49P) that causes familial encephalopathy with neuroserpin inclusion bodies is a poor proteinase inhibitor and readily forms polymers in vitro.

Belorgey D, Crowther DC, Mahadeva R, Lomas DA. Belorgey D, et al. Among authors: crowther dc. J Biol Chem. 2002 May 10;277(19):17367-73. doi: 10.1074/jbc.M200680200. Epub 2002 Mar 5. J Biol Chem. 2002. PMID: 11880376 Free article.

3

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. Miranda E, et al. Among authors: crowther dc. Hum Mol Genet. 2008 Jun 1;17(11):1527-39. doi: 10.1093/hmg/ddn041. Epub 2008 Feb 11. Hum Mol Genet. 2008. PMID: 18267959 Free PMC article.

4

Modeling serpin conformational diseases in Drosophila melanogaster.

Jahn TR, Malzer E, Roote J, Vishnivetskaya A, Imarisio S, Giannakou M, Panser K, Marciniak S, Crowther DC. Jahn TR, et al. Among authors: crowther dc. Methods Enzymol. 2011;499:227-58. doi: 10.1016/B978-0-12-386471-0.00012-2. Methods Enzymol. 2011. PMID: 21683257

5

Practical genetics: alpha-1-antitrypsin deficiency and the serpinopathies.

Crowther DC, Belorgey D, Miranda E, Kinghorn KJ, Sharp LK, Lomas DA. Crowther DC, et al. Eur J Hum Genet. 2004 Mar;12(3):167-72. doi: 10.1038/sj.ejhg.5201127. Eur J Hum Genet. 2004. PMID: 14694355 Review.

6

Sugar and alcohol molecules provide a therapeutic strategy for the serpinopathies that cause dementia and cirrhosis.

Sharp LK, Mallya M, Kinghorn KJ, Wang Z, Crowther DC, Huntington JA, Belorgey D, Lomas DA. Sharp LK, et al. Among authors: crowther dc. FEBS J. 2006 Jun;273(11):2540-52. doi: 10.1111/j.1742-4658.2006.05262.x. FEBS J. 2006. PMID: 16704419 Free article.

7

Polymerisation underlies alpha1-antitrypsin deficiency, dementia and other serpinopathies.

Lomas DA, Belorgey D, Mallya M, Onda M, Kinghorn KJ, Sharp LK, Phillips RL, Page R, Crowther DC, Miranda E. Lomas DA, et al. Among authors: crowther dc. Front Biosci. 2004 Sep 1;9:2873-91. doi: 10.2741/1444. Front Biosci. 2004. PMID: 15353322 Review.

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. Roussel BD, et al. Among authors: crowther dc. Hum Mol Genet. 2013 Nov 15;22(22):4616-26. doi: 10.1093/hmg/ddt310. Epub 2013 Jun 28. Hum Mol Genet. 2013. PMID: 23814041 Free PMC article.

9

Serpins: implications of a mobile reactive centre.

Crowther DC, Evans DL, Carrell RW. Crowther DC, et al. Curr Opin Biotechnol. 1992 Aug;3(4):399-407. doi: 10.1016/0958-1669(92)90169-j. Curr Opin Biotechnol. 1992. PMID: 1368442 Review.

10

Neuroserpin Portland (Ser52Arg) is trapped as an inactive intermediate that rapidly forms polymers: implications for the epilepsy seen in the dementia FENIB.

Belorgey D, Sharp LK, Crowther DC, Onda M, Johansson J, Lomas DA. Belorgey D, et al. Among authors: crowther dc. Eur J Biochem. 2004 Aug;271(16):3360-7. doi: 10.1111/j.1432-1033.2004.04270.x. Eur J Biochem. 2004. PMID: 15291813 Free article.

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