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Results: 1 to 20 of 21


A Huntingtin-based peptide inhibitor of caspase-6 provides protection from mutant Huntingtin-induced motor and behavioral deficits.

Aharony I, Ehrnhoefer DE, Shruster A, Qiu X, Franciosi S, Hayden MR, Offen D.

Hum Mol Genet. 2015 May 1;24(9):2604-14. doi: 10.1093/hmg/ddv023. Epub 2015 Jan 23.


Partial rescue of some features of Huntington Disease in the genetic absence of caspase-6 in YAC128 mice.

Wong BK, Ehrnhoefer DE, Graham RK, Martin DD, Ladha S, Uribe V, Stanek LM, Franciosi S, Qiu X, Deng Y, Kovalik V, Zhang W, Pouladi MA, Shihabuddin LS, Hayden MR.

Neurobiol Dis. 2015 Apr;76:24-36. doi: 10.1016/j.nbd.2014.12.030. Epub 2015 Jan 9.


Autophagy in Huntington disease and huntingtin in autophagy.

Martin DD, Ladha S, Ehrnhoefer DE, Hayden MR.

Trends Neurosci. 2015 Jan;38(1):26-35. doi: 10.1016/j.tins.2014.09.003. Epub 2014 Oct 2.


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.


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.


Intrinsic cleavage of receptor-interacting protein kinase-1 by caspase-6.

van Raam BJ, Ehrnhoefer DE, Hayden MR, Salvesen GS.

Cell Death Differ. 2013 Jan;20(1):86-96. doi: 10.1038/cdd.2012.98. Epub 2012 Aug 3.


NP03, a novel low-dose lithium formulation, is neuroprotective in the YAC128 mouse model of Huntington disease.

Pouladi MA, Brillaud E, Xie Y, Conforti P, Graham RK, Ehrnhoefer DE, Franciosi S, Zhang W, Poucheret P, Compte E, Maurel JC, Zuccato C, Cattaneo E, NĂ©ri C, Hayden MR.

Neurobiol Dis. 2012 Dec;48(3):282-9. doi: 10.1016/j.nbd.2012.06.026. Epub 2012 Jul 10.


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.


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.


Caspase-6 and neurodegeneration.

Graham RK, Ehrnhoefer DE, Hayden MR.

Trends Neurosci. 2011 Dec;34(12):646-56. doi: 10.1016/j.tins.2011.09.001. Epub 2011 Oct 22. Review.


Convergent pathogenic pathways in Alzheimer's and Huntington's diseases: shared targets for drug development.

Ehrnhoefer DE, Wong BK, Hayden MR.

Nat Rev Drug Discov. 2011 Oct 21;10(11):853-67. doi: 10.1038/nrd3556. Review.


Mice lacking caspase-2 are protected from behavioral changes, but not pathology, in the YAC128 model of Huntington disease.

Carroll JB, Southwell AL, Graham RK, Lerch JP, Ehrnhoefer DE, Cao LP, Zhang WN, Deng Y, Bissada N, Henkelman RM, Hayden MR.

Mol Neurodegener. 2011 Aug 19;6:59. doi: 10.1186/1750-1326-6-59.


Small changes, big impact: posttranslational modifications and function of huntingtin in Huntington disease.

Ehrnhoefer DE, Sutton L, Hayden MR.

Neuroscientist. 2011 Oct;17(5):475-92. doi: 10.1177/1073858410390378. Epub 2011 Feb 10. Review.


EGCG remodels mature alpha-synuclein and amyloid-beta fibrils and reduces cellular toxicity.

Bieschke J, Russ J, Friedrich RP, Ehrnhoefer DE, Wobst H, Neugebauer K, Wanker EE.

Proc Natl Acad Sci U S A. 2010 Apr 27;107(17):7710-5. doi: 10.1073/pnas.0910723107. Epub 2010 Apr 12.


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.


Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin.

Okamoto S, Pouladi MA, Talantova M, Yao D, Xia P, Ehrnhoefer DE, Zaidi R, Clemente A, Kaul M, Graham RK, Zhang D, Vincent Chen HS, Tong G, Hayden MR, Lipton SA.

Nat Med. 2009 Dec;15(12):1407-13. doi: 10.1038/nm.2056. Epub 2009 Nov 15.


MCAD mutations identified in newborn screening cause different levels of enzymatic dysfunction.

Ehrnhoefer DE.

Clin Genet. 2009 Aug;76(2):146-8. doi: 10.1111/j.1399-0004.2009.01247_1.x. Epub 2009 Aug 7. No abstract available.


IFRD1 modulates disease severity in cystic fibrosis through the regulation of neutrophil effector function.

Ehrnhoefer DE.

Clin Genet. 2009 Aug;76(2):148-9. doi: 10.1111/j.1399-0004.2009.01247_2.x. Epub 2009 Aug 7. No abstract available.


Mouse models of Huntington disease: variations on a theme.

Ehrnhoefer DE, Butland SL, Pouladi MA, Hayden MR.

Dis Model Mech. 2009 Mar-Apr;2(3-4):123-9. doi: 10.1242/dmm.002451.


EGCG redirects amyloidogenic polypeptides into unstructured, off-pathway oligomers.

Ehrnhoefer DE, Bieschke J, Boeddrich A, Herbst M, Masino L, Lurz R, Engemann S, Pastore A, Wanker EE.

Nat Struct Mol Biol. 2008 Jun;15(6):558-66. doi: 10.1038/nsmb.1437. Epub 2008 May 30.

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