Walkley SU - Search Results

1

Cerebellar alterations and gait defects as therapeutic outcome measures for enzyme replacement therapy in α-mannosidosis.

Damme M, Stroobants S, Walkley SU, Lüllmann-Rauch R, D'Hooge R, Fogh J, Saftig P, Lübke T, Blanz J. Damme M, et al. Among authors: walkley su. J Neuropathol Exp Neurol. 2011 Jan;70(1):83-94. doi: 10.1097/NEN.0b013e31820428fa. J Neuropathol Exp Neurol. 2011. PMID: 21157375 Free PMC article.

2

Ataxia is the major neuropathological finding in arylsulfatase G-deficient mice: similarities and dissimilarities to Sanfilippo disease (mucopolysaccharidosis type III).

Kowalewski B, Heimann P, Ortkras T, Lüllmann-Rauch R, Sawada T, Walkley SU, Dierks T, Damme M. Kowalewski B, et al. Among authors: walkley su. Hum Mol Genet. 2015 Apr 1;24(7):1856-68. doi: 10.1093/hmg/ddu603. Epub 2014 Dec 1. Hum Mol Genet. 2015. PMID: 25452429 Free PMC article.

3

Acid Ceramidase Deficiency in Mice Results in a Broad Range of Central Nervous System Abnormalities.

Sikora J, Dworski S, Jones EE, Kamani MA, Micsenyi MC, Sawada T, Le Faouder P, Bertrand-Michel J, Dupuy A, Dunn CK, Xuan ICY, Casas J, Fabrias G, Hampson DR, Levade T, Drake RR, Medin JA, Walkley SU. Sikora J, et al. Among authors: walkley su. Am J Pathol. 2017 Apr;187(4):864-883. doi: 10.1016/j.ajpath.2016.12.005. Am J Pathol. 2017. PMID: 28342444 Free PMC article.

4

Cyclodextrin has conflicting actions on autophagy flux in vivo in brains of normal and Alzheimer model mice.

Yang DS, Stavrides P, Kumar A, Jiang Y, Mohan PS, Ohno M, Dobrenis K, Davidson CD, Saito M, Pawlik M, Huo C, Walkley SU, Nixon RA. Yang DS, et al. Among authors: walkley su. Hum Mol Genet. 2017 Mar 1;26(5):843-859. doi: 10.1093/hmg/ddx001. Hum Mol Genet. 2017. PMID: 28062666 Free PMC article.

5

X-linked Christianson syndrome: heterozygous female Slc9a6 knockout mice develop mosaic neuropathological changes and related behavioral abnormalities.

Sikora J, Leddy J, Gulinello M, Walkley SU. Sikora J, et al. Among authors: walkley su. Dis Model Mech. 2016 Jan;9(1):13-23. doi: 10.1242/dmm.022780. Epub 2015 Oct 29. Dis Model Mech. 2016. PMID: 26515654 Free PMC article.

6

Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport.

Sleat DE, Wiseman JA, El-Banna M, Price SM, Verot L, Shen MM, Tint GS, Vanier MT, Walkley SU, Lobel P. Sleat DE, et al. Among authors: walkley su. Proc Natl Acad Sci U S A. 2004 Apr 20;101(16):5886-91. doi: 10.1073/pnas.0308456101. Epub 2004 Apr 7. Proc Natl Acad Sci U S A. 2004. PMID: 15071184 Free PMC article.

7

Microarray expression analysis and identification of serum biomarkers for Niemann-Pick disease, type C1.

Cluzeau CV, Watkins-Chow DE, Fu R, Borate B, Yanjanin N, Dail MK, Davidson CD, Walkley SU, Ory DS, Wassif CA, Pavan WJ, Porter FD. Cluzeau CV, et al. Among authors: walkley su. Hum Mol Genet. 2012 Aug 15;21(16):3632-46. doi: 10.1093/hmg/dds193. Epub 2012 May 22. Hum Mol Genet. 2012. PMID: 22619379 Free PMC article.

8

N-butyldeoxynojirimycin delays motor deficits, cerebellar microgliosis, and Purkinje cell loss in a mouse model of mucolipidosis type IV.

Boudewyn LC, Sikora J, Kuchar L, Ledvinova J, Grishchuk Y, Wang SL, Dobrenis K, Walkley SU. Boudewyn LC, et al. Among authors: walkley su. Neurobiol Dis. 2017 Sep;105:257-270. doi: 10.1016/j.nbd.2017.06.003. Epub 2017 Jun 10. Neurobiol Dis. 2017. PMID: 28610891 Free PMC article.

9

Intracisternal cyclodextrin prevents cerebellar dysfunction and Purkinje cell death in feline Niemann-Pick type C1 disease.

Vite CH, Bagel JH, Swain GP, Prociuk M, Sikora TU, Stein VM, O'Donnell P, Ruane T, Ward S, Crooks A, Li S, Mauldin E, Stellar S, De Meulder M, Kao ML, Ory DS, Davidson C, Vanier MT, Walkley SU. Vite CH, et al. Among authors: walkley su. Sci Transl Med. 2015 Feb 25;7(276):276ra26. doi: 10.1126/scitranslmed.3010101. Sci Transl Med. 2015. PMID: 25717099 Free PMC article.

10

A murine Niemann-Pick C1 I1061T knock-in model recapitulates the pathological features of the most prevalent human disease allele.

Praggastis M, Tortelli B, Zhang J, Fujiwara H, Sidhu R, Chacko A, Chen Z, Chung C, Lieberman AP, Sikora J, Davidson C, Walkley SU, Pipalia NH, Maxfield FR, Schaffer JE, Ory DS. Praggastis M, et al. Among authors: walkley su. J Neurosci. 2015 May 27;35(21):8091-106. doi: 10.1523/JNEUROSCI.4173-14.2015. J Neurosci. 2015. PMID: 26019327 Free PMC article.

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