Weimer JM - Search Results

1

Genetic ataxia telangiectasia porcine model phenocopies the multisystemic features of the human disease.

Beraldi R, Meyerholz DK, Savinov A, Kovács AD, Weimer JM, Dykstra JA, Geraets RD, Pearce DA. Beraldi R, et al. Among authors: weimer jm. Biochim Biophys Acta Mol Basis Dis. 2017 Nov;1863(11):2862-2870. doi: 10.1016/j.bbadis.2017.07.020. Epub 2017 Jul 23. Biochim Biophys Acta Mol Basis Dis. 2017. PMID: 28746835 Free PMC article.

2

The neuronal ceroid lipofuscinoses: mutations in different proteins result in similar disease.

Weimer JM, Kriscenski-Perry E, Elshatory Y, Pearce DA. Weimer JM, et al. Neuromolecular Med. 2002;1(2):111-24. doi: 10.1385/NMM:1:2:111. Neuromolecular Med. 2002. PMID: 12025857 Review.

3

CLN3, the protein associated with batten disease: structure, function and localization.

Phillips SN, Benedict JW, Weimer JM, Pearce DA. Phillips SN, et al. Among authors: weimer jm. J Neurosci Res. 2005 Mar 1;79(5):573-83. doi: 10.1002/jnr.20367. J Neurosci Res. 2005. PMID: 15657902 Review.

4

Elevation of Hook1 in a disease model of Batten disease does not affect a novel interaction between Ankyrin G and Hook1.

Weimer JM, Chattopadhyay S, Custer AW, Pearce DA. Weimer JM, et al. Biochem Biophys Res Commun. 2005 May 20;330(4):1176-81. doi: 10.1016/j.bbrc.2005.03.103. Biochem Biophys Res Commun. 2005. PMID: 15823567

5

Novel CLN3 mutation predicted to cause complete loss of protein function does not modify the classical JNCL phenotype.

Kwon JM, Rothberg PG, Leman AR, Weimer JM, Mink JW, Pearce DA. Kwon JM, et al. Among authors: weimer jm. Neurosci Lett. 2005 Oct 21;387(2):111-4. doi: 10.1016/j.neulet.2005.07.023. Neurosci Lett. 2005. PMID: 16087292

6

Visual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neurons.

Weimer JM, Custer AW, Benedict JW, Alexander NA, Kingsley E, Federoff HJ, Cooper JD, Pearce DA. Weimer JM, et al. Neurobiol Dis. 2006 May;22(2):284-93. doi: 10.1016/j.nbd.2005.11.008. Epub 2006 Jan 18. Neurobiol Dis. 2006. PMID: 16412658 Free PMC article.

7

Selectively increased sensitivity of cerebellar granule cells to AMPA receptor-mediated excitotoxicity in a mouse model of Batten disease.

Kovács AD, Weimer JM, Pearce DA. Kovács AD, et al. Among authors: weimer jm. Neurobiol Dis. 2006 Jun;22(3):575-85. doi: 10.1016/j.nbd.2005.12.018. Epub 2006 Feb 17. Neurobiol Dis. 2006. PMID: 16483786

8

Alterations in striatal dopamine catabolism precede loss of substantia nigra neurons in a mouse model of juvenile neuronal ceroid lipofuscinosis.

Weimer JM, Benedict JW, Elshatory YM, Short DW, Ramirez-Montealegre D, Ryan DA, Alexander NA, Federoff HJ, Cooper JD, Pearce DA. Weimer JM, et al. Brain Res. 2007 Aug 8;1162:98-112. doi: 10.1016/j.brainres.2007.05.018. Epub 2007 May 21. Brain Res. 2007. PMID: 17617387 Free PMC article.

9

Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis.

Weimer JM, Benedict JW, Getty AL, Pontikis CC, Lim MJ, Cooper JD, Pearce DA. Weimer JM, et al. Brain Res. 2009 Apr 17;1266:93-107. doi: 10.1016/j.brainres.2009.02.009. Epub 2009 Feb 20. Brain Res. 2009. PMID: 19230832 Free PMC article.

10

A novel porcine model of ataxia telangiectasia reproduces neurological features and motor deficits of human disease.

Beraldi R, Chan CH, Rogers CS, Kovács AD, Meyerholz DK, Trantzas C, Lambertz AM, Darbro BW, Weber KL, White KA, Rheeden RV, Kruer MC, Dacken BA, Wang XJ, Davis BT, Rohret JA, Struzynski JT, Rohret FA, Weimer JM, Pearce DA. Beraldi R, et al. Among authors: weimer jm. Hum Mol Genet. 2015 Nov 15;24(22):6473-84. doi: 10.1093/hmg/ddv356. Epub 2015 Sep 15. Hum Mol Genet. 2015. PMID: 26374845 Free PMC article.

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