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Items: 23

1.

Activation of Caspase-6 Is Promoted by a Mutant Huntingtin Fragment and Blocked by an Allosteric Inhibitor Compound.

Ehrnhoefer DE, Skotte NH, Reinshagen J, Qiu X, Windshügel B, Jaishankar P, Ladha S, Petina O, Khankischpur M, Nguyen YTN, Caron NS, Razeto A, Meyer Zu Rheda M, Deng Y, Huynh KT, Wittig I, Gribbon P, Renslo AR, Geffken D, Gul S, Hayden MR.

Cell Chem Biol. 2019 Jul 15. pii: S2451-9456(19)30209-0. doi: 10.1016/j.chembiol.2019.07.001. [Epub ahead of print]

PMID:
31353319
2.

Functional Differences between Synaptic Mitochondria from the Striatum and the Cerebral Cortex.

Petersen MH, Willert CW, Andersen JV, Waagepetersen HS, Skotte NH, Nørremølle A.

Neuroscience. 2019 May 15;406:432-443. doi: 10.1016/j.neuroscience.2019.02.033. Epub 2019 Mar 13.

PMID:
30876983
3.

Enhanced cerebral branched-chain amino acid metabolism in R6/2 mouse model of Huntington's disease.

Andersen JV, Skotte NH, Aldana BI, Nørremølle A, Waagepetersen HS.

Cell Mol Life Sci. 2019 Jun;76(12):2449-2461. doi: 10.1007/s00018-019-03051-2. Epub 2019 Mar 4.

PMID:
30830240
4.

Huntingtin suppression restores cognitive function in a mouse model of Huntington's disease.

Southwell AL, Kordasiewicz HB, Langbehn D, Skotte NH, Parsons MP, Villanueva EB, Caron NS, Østergaard ME, Anderson LM, Xie Y, Cengio LD, Findlay-Black H, Doty CN, Fitsimmons B, Swayze EE, Seth PP, Raymond LA, Frank Bennett C, Hayden MR.

Sci Transl Med. 2018 Oct 3;10(461). pii: eaar3959. doi: 10.1126/scitranslmed.aar3959.

PMID:
30282695
5.

Site-specific characterization of endogenous SUMOylation across species and organs.

Hendriks IA, Lyon D, Su D, Skotte NH, Daniel JA, Jensen LJ, Nielsen ML.

Nat Commun. 2018 Jun 25;9(1):2456. doi: 10.1038/s41467-018-04957-4.

6.

Integrative Characterization of the R6/2 Mouse Model of Huntington's Disease Reveals Dysfunctional Astrocyte Metabolism.

Skotte NH, Andersen JV, Santos A, Aldana BI, Willert CW, Nørremølle A, Waagepetersen HS, Nielsen ML.

Cell Rep. 2018 May 15;23(7):2211-2224. doi: 10.1016/j.celrep.2018.04.052.

7.

Preventing mutant huntingtin proteolysis and intermittent fasting promote autophagy in models of Huntington disease.

Ehrnhoefer DE, Martin DDO, Schmidt ME, Qiu X, Ladha S, Caron NS, Skotte NH, Nguyen YTN, Vaid K, Southwell AL, Engemann S, Franciosi S, Hayden MR.

Acta Neuropathol Commun. 2018 Mar 6;6(1):16. doi: 10.1186/s40478-018-0518-0.

8.

A novel humanized mouse model of Huntington disease for preclinical development of therapeutics targeting mutant huntingtin alleles.

Southwell AL, Skotte NH, Villanueva EB, Østergaard ME, Gu X, Kordasiewicz HB, Kay C, Cheung D, Xie Y, Waltl S, Dal Cengio L, Findlay-Black H, Doty CN, Petoukhov E, Iworima D, Slama R, Ooi J, Pouladi MA, Yang XW, Swayze EE, Seth PP, Hayden MR.

Hum Mol Genet. 2017 Mar 15;26(6):1115-1132. doi: 10.1093/hmg/ddx021.

PMID:
28104789
9.

Palmitoylation of caspase-6 by HIP14 regulates its activation.

Skotte NH, Sanders SS, Singaraja RR, Ehrnhoefer DE, Vaid K, Qiu X, Kannan S, Verma C, Hayden MR.

Cell Death Differ. 2017 Mar;24(3):433-444. doi: 10.1038/cdd.2016.139. Epub 2016 Dec 2.

10.

Huntingtin Haplotypes Provide Prioritized Target Panels for Allele-specific Silencing in Huntington Disease Patients of European Ancestry.

Kay C, Collins JA, Skotte NH, Southwell AL, Warby SC, Caron NS, Doty CN, Nguyen B, Griguoli A, Ross CJ, Squitieri F, Hayden MR.

Mol Ther. 2015 Nov;23(11):1759-1771. doi: 10.1038/mt.2015.128. Epub 2015 Jul 23.

11.

Allele-specific suppression of mutant huntingtin using antisense oligonucleotides: providing a therapeutic option for all Huntington disease patients.

Skotte NH, Southwell AL, Østergaard ME, Carroll JB, Warby SC, Doty CN, Petoukhov E, Vaid K, Kordasiewicz H, Watt AT, Freier SM, Hung G, Seth PP, Bennett CF, Swayze EE, Hayden MR.

PLoS One. 2014 Sep 10;9(9):e107434. doi: 10.1371/journal.pone.0107434. eCollection 2014.

12.

In vivo evaluation of candidate allele-specific mutant huntingtin gene silencing antisense oligonucleotides.

Southwell AL, Skotte NH, Kordasiewicz HB, Østergaard ME, Watt AT, Carroll JB, Doty CN, Villanueva EB, Petoukhov E, Vaid K, Xie Y, Freier SM, Swayze EE, Seth PP, Bennett CF, Hayden MR.

Mol Ther. 2014 Dec;22(12):2093-106. doi: 10.1038/mt.2014.153. Epub 2014 Aug 7.

13.

Personalized gene silencing therapeutics for Huntington disease.

Kay C, Skotte NH, Southwell AL, Hayden MR.

Clin Genet. 2014 Jul;86(1):29-36. doi: 10.1111/cge.12385. Epub 2014 Apr 11. Review.

PMID:
24646433
14.

HACE1 reduces oxidative stress and mutant Huntingtin toxicity by promoting the NRF2 response.

Rotblat B, Southwell AL, Ehrnhoefer DE, Skotte NH, Metzler M, Franciosi S, Leprivier G, Somasekharan SP, Barokas A, Deng Y, Tang T, Mathers J, Cetinbas N, Daugaard M, Kwok B, Li L, Carnie CJ, Fink D, Nitsch R, Galpin JD, Ahern CA, Melino G, Penninger JM, Hayden MR, Sorensen PH.

Proc Natl Acad Sci U S A. 2014 Feb 25;111(8):3032-7. doi: 10.1073/pnas.1314421111. Epub 2014 Feb 10.

15.

p53 increases caspase-6 expression and activation in muscle tissue expressing mutant huntingtin.

Ehrnhoefer DE, Skotte NH, Ladha S, Nguyen YT, Qiu X, Deng Y, Huynh KT, Engemann S, Nielsen SM, Becanovic K, Leavitt BR, Hasholt L, Hayden MR.

Hum Mol Genet. 2014 Feb 1;23(3):717-29. doi: 10.1093/hmg/ddt458. Epub 2013 Sep 18.

PMID:
24070868
16.

Rational design of antisense oligonucleotides targeting single nucleotide polymorphisms for potent and allele selective suppression of mutant Huntingtin in the CNS.

Østergaard ME, Southwell AL, Kordasiewicz H, Watt AT, Skotte NH, Doty CN, Vaid K, Villanueva EB, Swayze EE, Bennett CF, Hayden MR, Seth PP.

Nucleic Acids Res. 2013 Nov;41(21):9634-50. doi: 10.1093/nar/gkt725. Epub 2013 Aug 19.

17.

Antisense oligonucleotide therapeutics for inherited neurodegenerative diseases.

Southwell AL, Skotte NH, Bennett CF, Hayden MR.

Trends Mol Med. 2012 Nov;18(11):634-43. doi: 10.1016/j.molmed.2012.09.001. Epub 2012 Sep 28. Review.

PMID:
23026741
18.

A fully humanized transgenic mouse model of Huntington disease.

Southwell AL, Warby SC, Carroll JB, Doty CN, Skotte NH, Zhang W, Villanueva EB, Kovalik V, Xie Y, Pouladi MA, Collins JA, Yang XW, Franciosi S, Hayden MR.

Hum Mol Genet. 2013 Jan 1;22(1):18-34. doi: 10.1093/hmg/dds397. Epub 2012 Sep 21.

19.

Rescue from excitotoxicity and axonal degeneration accompanied by age-dependent behavioral and neuroanatomical alterations in caspase-6-deficient mice.

Uribe V, Wong BK, Graham RK, Cusack CL, Skotte NH, Pouladi MA, Xie Y, Feinberg K, Ou Y, Ouyang Y, Deng Y, Franciosi S, Bissada N, Spreeuw A, Zhang W, Ehrnhoefer DE, Vaid K, Miller FD, Deshmukh M, Howland D, Hayden MR.

Hum Mol Genet. 2012 May 1;21(9):1954-67. doi: 10.1093/hmg/dds005. Epub 2012 Jan 18.

20.

A quantitative method for the specific assessment of caspase-6 activity in cell culture.

Ehrnhoefer DE, Skotte NH, Savill J, Nguyen YT, Ladha S, Cao LP, Dullaghan E, Hayden MR.

PLoS One. 2011;6(11):e27680. doi: 10.1371/journal.pone.0027680. Epub 2011 Nov 29.

21.

Polycystin-1: a key player in hereditary cystic kidney and liver disorders.

Skotte NH.

Clin Genet. 2011 Dec;80(6):507-9. doi: 10.1111/j.1399-0004.2011.01780.x. Epub 2011 Sep 23. No abstract available.

PMID:
21895640
22.

Reduced gluconeogenesis and lactate clearance in Huntington's disease.

Josefsen K, Nielsen SM, Campos A, Seifert T, Hasholt L, Nielsen JE, Nørremølle A, Skotte NH, Secher NH, Quistorff B.

Neurobiol Dis. 2010 Dec;40(3):656-62. doi: 10.1016/j.nbd.2010.08.009. Epub 2010 Aug 19.

PMID:
20727971
23.

Full-length huntingtin levels modulate body weight by influencing insulin-like growth factor 1 expression.

Pouladi MA, Xie Y, Skotte NH, Ehrnhoefer DE, Graham RK, Kim JE, Bissada N, Yang XW, Paganetti P, Friedlander RM, Leavitt BR, Hayden MR.

Hum Mol Genet. 2010 Apr 15;19(8):1528-38. doi: 10.1093/hmg/ddq026. Epub 2010 Jan 22.

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