Neurobiology and cellular pathogenesis of glycolipid storage diseases.

Yazar: Walkley, Steven U
Baskı/Yayın Bilgisi 2003

Pathogenic Cascades in Lysosomal Disease – Why so Complex?

Yazar: Walkley, Steven U.
Baskı/Yayın Bilgisi 2009

Cellular Pathology of Lysosomal Storage Disorders

Yazar: Walkley, Steven U.
Yayımlandı Brain Pathol (2006)

Pathomechanisms in Lysosomal Storage Disorders

Yazar: Walkley, Steven U., Vanier, Marie T.
Yayımlandı Biochim Biophys Acta (2008)

Current concepts in the neuropathogenesis of mucolipidosis type IV

Yazar: Boudewyn, Lauren C., Walkley, Steven U.
Yayımlandı J Neurochem (2018)

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

Yazar: Sikora, Jakub, Leddy, Jennifer, Gulinello, Maria, Walkley, Steven U.
Yayımlandı Dis Model Mech (2016)

Lysosomal Compromise and Brain Dysfunction: Examining the Role of Neuroaxonal Dystrophy

Yazar: Walkley, Steven U., Sikora, Jakub, Micsenyi, Matthew, Davidson, Cristin, Dobrenis, Kostantin
Yayımlandı Biochem Soc Trans (2010)

Lysosomal Membrane Permeability Stimulates Protein Aggregate Formation in Neurons of a Lysosomal Disease

Yazar: Micsenyi, Matthew C., Sikora, Jakub, Stephney, Gloria, Dobrenis, Kostantin, Walkley, Steven U.
Baskı/Yayın Bilgisi 2013

Autophagy in lysosomal storage disorders

Yazar: Lieberman, Andrew P., Puertollano, Rosa, Raben, Nina, Slaugenhaupt, Susan, Walkley, Steven U., Ballabio, Andrea
Baskı/Yayın Bilgisi 2012

Cyclodextrin alleviates neuronal storage of cholesterol in Niemann-Pick C disease without evidence of detectable blood-brain barrier permeability

Yazar: Pontikis, Charles C., Davidson, Cristin D., Walkley, Steven U., Platt, Frances M., Begley, David J
Baskı/Yayın Bilgisi 2013

Endosomal/Lysosomal Processing of Gangliosides Affects Neuronal Cholesterol Sequestration in Niemann-Pick Disease Type C

Yazar: Zhou, Sharon, Davidson, Cristin, McGlynn, Robert, Stephney, Gloria, Dobrenis, Kostantin, Vanier, Marie T., Walkley, Steven U.
Baskı/Yayın Bilgisi 2011

Atypical multisensory integration in Niemann-Pick type C disease – towards potential biomarkers

Yazar: Andrade, Gizely N, Molholm, Sophie, Butler, John S, Brandwein, Alice B, Walkley, Steven U, Foxe, John J
Baskı/Yayın Bilgisi 2014

Neuropathology of the Mcoln1(−/−) Knockout Mouse Model of Mucolipidosis Type IV

Yazar: Micsenyi, Matthew C., Dobrenis, Kostantin, Stephney, Gloria, Pickel, James, Vanier, Marie T., Slaugenhaupt, Susan A., Walkley, Steven U.
Yayımlandı J Neuropathol Exp Neurol (2009)

Residual levels of tripeptidyl-peptidase I activity dramatically ameliorate disease in late infantile neuronal ceroid lipofuscinosis

Yazar: Sleat, David E., El-Banna, Mukarram, Sohar, Istvan, Kim, Kwi-Hye, Dobrenis, Kostantin, Walkley, Steven U., Lobel, Peter
Baskı/Yayın Bilgisi 2008

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

Yazar: Kowalewski, Björn, Heimann, Peter, Ortkras, Theresa, Lüllmann-Rauch, Renate, Sawada, Tomo, Walkley, Steven U., Dierks, Thomas, Damme, Markus
Yayımlandı Hum Mol Genet (2015)

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

Yazar: Boudewyn, Lauren C., Sikora, Jakub, Kuchar, Ladislav, Ledvinova, Jana, Grishchuk, Yulia, Wang, Shirley L., Dobrenis, Kostantin, Walkley, Steven U.
Yayımlandı Neurobiol Dis (2017)

Free Sialic Acid Storage Disorder: Progress and Promise

Yazar: Huizing, Marjan, Hackbarth, Mary E., Adams, David R, Wasserstein, Melissa, Patterson, Marc C, Walkley, Steven U, Gahl, William A
Yayımlandı Neurosci Lett (2021)

Cerebellar Alterations and Gait Defects as Therapeutic Outcome Measures for Enzyme Replacement Therapy in α-Mannosidosis

Yazar: Damme, Markus, Stroobants, Stijn, Walkley, Steven U., Lüllmann-Rauch, Renate, D`Hooge, Rudi, Fogh, Jens, Saftig, Paul, Lübke, Torben, Blanz, Judith
Baskı/Yayın Bilgisi 2011

Neurologic, Gastric, and Opthalmologic Pathologies in a Murine Model of Mucolipidosis Type IV

Yazar: Venugopal, Bhuvarahamurthy , Browning, Marsha F. , Curcio-Morelli, Cyntia , Varro, Andrea , Michaud, Norman , Nanthakumar, Nanda , Walkley, Steven U. , Pickel, James , Slaugenhaupt, Susan A. 
Baskı/Yayın Bilgisi 2007

Chronic Cyclodextrin Treatment of Murine Niemann-Pick C Disease Ameliorates Neuronal Cholesterol and Glycosphingolipid Storage and Disease Progression

Yazar: Davidson, Cristin D., Ali, Nafeeza F., Micsenyi, Matthew C., Stephney, Gloria, Renault, Sophie, Dobrenis, Kostantin, Ory, Daniel S., Vanier, Marie T., Walkley, Steven U.
Baskı/Yayın Bilgisi 2009