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1

Generation of a Novel Mouse Model that Mimics Farber Disease

Abdu Alayoubi, James Wang, Bryan Au, Stéphane Carpentier, Virginie Garcia, Shaalee Dworski, Samah El-Ghmrsni, Razq Hakem, Thierry Levade, Jeffrey Medin

Journal:

Molecular Genetics and Metabolism

Année:

2012

Langue:

english

Fichier:

PDF, 58 KB

english, 2012

2

Gene Expression Profiling of a Mouse Model of Fabry Disease

Shaalee Dworski, Salvador Mejia-Guerrero, Natalia Pacienza, Bryan Au, Jeffrey Medin

Journal:

Molecular Genetics and Metabolism

Année:

2012

Langue:

english

Fichier:

PDF, 56 KB

english, 2012

3

179 THE DYNAMIC BLADDER MICROENVIRONMENT GOVERNS SMOOTH MUSCLE DIFFERENTIATION OF SKIN DERIVED PRECURSOR CELLS: IMPLICATIONS FOR TISSUE REGENERATION

Tolg, Cornelia, Dworski, Shaalee, Biernaskie, Jeff, Aitken, Karen, Miller, Freda, Bagli, Darius

Journal:

The Journal of Urology

Année:

2011

Langue:

english

Fichier:

PDF, 76 KB

english, 2011

4

201 BLADDER STRAIN INDUCED MICROENVIRONMENT ATTENUATES SMOOTH MUSCLE DIFFERENTIATION OF ADULT RAT SKIN DERIVED PRECURSOR CELLS: IMPLICATIONS FOR TISSUE REGENERATION/ENGINEERING

Tolg, Cornelia, Dworski, Shaalee, Aitken, Karen, Biernaskie, Jeff, Bagli, Darius

Journal:

The Journal of Urology

Année:

2012

Langue:

english

Fichier:

PDF, 148 KB

english, 2012

5

Hematopoiesis is dysregulated in a novel mouse model of Farber disease

Dworski, Shaalee, Berger, Alexandra, Yoshimitsu, Makoto, Alayoubi, Abdulfatah, Au, Bryan, Forster, Jessa, Furlonger, Caren, Wang, James C.M., Takenaka, Toshihiro, Paige, Christopher J., Medin, Jeffrey

Journal:

Molecular Genetics and Metabolism

Année:

2013

Langue:

english

Fichier:

PDF, 59 KB

english, 2013

6

Farber disease: characterization of behavior and brain pathology in a new murine model

Sawada, Tomo, Dworski, Shaalee, Sikora, Jakub, Micsenyi, Matthew, Medin, Jeffrey A., Walkley, Steven U., Hampson, David R.

Journal:

Molecular Genetics and Metabolism

Année:

2014

Langue:

english

Fichier:

PDF, 47 KB

english, 2014

7

87:6418 Estimating periphyton growth parameters using simple models

Sawada, Tomo, Dworski, Shaalee, Sikora, Jakub, Micsenyi, Matthew, Medin, Jeffrey A., Walkley, Steven U., Hampson, David R.

Journal:

Deep Sea Research Part B. Oceanographic Literature Review

Année:

1987

Langue:

english

Fichier:

PDF, 90 KB

english, 1987

8

On-tissue profiling of ceramides and other sphingolipids in lysosomal disorders using high resolution MALDI-imaging mass spectrometry

Jones, E. Ellen, Dworski, Shaalee, Kamani, Mustafa, Medin, Jeffrey A., Drake, Richard R.

Journal:

Molecular Genetics and Metabolism

Année:

2014

Langue:

english

Fichier:

PDF, 190 KB

english, 2014

9

Altered MCP-1 and ceramide metabolite levels in serum from Farber mice and Farber patients

Dworski, Shaalee, Lu, Ping, Calatayud, Anna-Line, Maranda, Bruno, Mitchell, John, Khan, Aneal, Levade, Thierry, Norris, James S., Medin, Jeffrey A.

Journal:

Molecular Genetics and Metabolism

Année:

2014

Langue:

english

Fichier:

PDF, 45 KB

english, 2014

10

Direct Genesis of Functional Rodent and Human Schwann Cells from Skin Mesenchymal Precursors

Krause, Matthew P., Dworski, Shaalee, Feinberg, Konstantin, Jones, Karen, Johnston, Adam P.W., Paul, Smitha, Paris, Maryline, Peles, Elior, Bagli, Darius, Forrest, Christopher R., Kaplan, David R., Mi

Journal:

Stem Cell Reports

Année:

2014

Langue:

english

Fichier:

PDF, 6.39 MB

english, 2014

11

On-Tissue Localization of Ceramides and Other Sphingolipids by MALDI Mass Spectrometry Imaging

Jones, E. Ellen, Dworski, Shaalee, Canals, Daniel, Casas, Josefina, Fabrias, Gemma, Schoenling, Drew, Levade, Thierry, Denlinger, Chadrick, Hannun, Yusuf A., Medin, Jeffrey A., Drake, Richard R.

Journal:

Analytical Chemistry

Année:

2014

Langue:

english

Fichier:

PDF, 4.91 MB

english, 2014

12

Profiling of glycosphingolipids for relative organ distribution in a pure strain of Fabry mice

Provençal, Philippe, Boutin, Michel, Au, Bryan, Dworski, Shaalee, Medin, Jeffrey A., Auray-Blais, Christiane

Journal:

Clinical Biochemistry

Année:

2014

Langue:

english

Fichier:

PDF, 45 KB

english, 2014

13

Systemic ceramide accumulation leads to severe and varied pathological consequences

Alayoubi, Abdulfatah M., Wang, James C. M., Au, Bryan C. Y., Carpentier, Stéphane, Garcia, Virginie, Dworski, Shaalee, El-Ghamrasni, Samah, Kirouac, Kevin N., Exertier, Mathilde J., Xiong, Zi Jian, Pr

Journal:

EMBO Molecular Medicine

Année:

2013

Langue:

english

Fichier:

PDF, 2.00 MB

english, 2013

14

Spatial distribution of brain ceramides in an acid ceramidase deficient murine model: Subsequent histological manifestations and functional deficits

Dworski, Shaalee, Jones, Elizabeth E., Sikora, Jakub, Micsenyi, Matthew, Sawada, Tomo, Le Faouder, Pauline, Bertrand-Michel, Justine, Dupuy, Aude, Kamani, Mustafa, Dunn, Christopher K., Xuan, Ingrid,

Journal:

Molecular Genetics and Metabolism

Année:

2015

Langue:

english

Fichier:

PDF, 407 KB

english, 2015

15

Acid ceramidase deficiency leads to multiple skin abnormalities in a mouse model of Farber disease

Lopez-Vasquez, Lucia, Dworski, Shaalee, Pouliot, Roxane, Galbraith, Todd, Kamani, Mustafa A., Lacroix, Dan, Auger, François A., Medin, Jeffrey A.

Journal:

Molecular Genetics and Metabolism

Année:

2016

Langue:

english

Fichier:

PDF, 74 KB

english, 2016

16

Relative distribution of Gb 3 isoforms/analogs in NOD/SCID/Fabry mice tissues determined by tandem mass spectrometry

Provençal, Philippe, Boutin, Michel, Dworski, Shaalee, Au, Bryan, Medin, Jeffrey A, Auray-Blais, Christiane

Journal:

Année:

2016

Langue:

english

Fichier:

PDF, 1.53 MB

english, 2016

17

Acid Ceramidase Deficiency is characterized by a unique plasma cytokine and ceramide profile that is altered by therapy

Dworski, Shaalee, Lu, Ping, Khan, Aneal, Maranda, Bruno, Mitchell, John J., Parini, Rossella, Di Rocco, Maja, Hugle, Boris, Yoshimitsu, Makoto, Magnusson, Bo, Makay, Balahan, Arslan, Nur, Guelbert, No

Journal:

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease

Année:

2017

Langue:

english

Fichier:

PDF, 2.02 MB

english, 2017

18

Enzyme replacement therapy for Farber disease: Proof-of-concept studies in cells and mice

He, Xingxuan, Dworski, Shaalee, Zhu, Changzhi, DeAngelis, Victor, Solyom, Alex, Medin, Jeffrey A., Simonaro, Calogera M., Schuchman, Edward H.

Journal:

Année:

2017

Langue:

english

Fichier:

PDF, 6.33 MB

english, 2017

19

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

Sikora, Jakub, Dworski, Shaalee, Jones, E. Ellen, Kamani, Mustafa A., Micsenyi, Matthew C., Sawada, Tomo, Le Faouder, Pauline, Bertrand-Michel, Justine, Dupuy, Aude, Dunn, Christopher K., Xuan, Ingrid

Journal:

The American Journal of Pathology

Année:

2017

Langue:

english

Fichier:

PDF, 8.02 MB

english, 2017

20

Proof-of-concept studies underlying enzyme replacement therapy for acid ceramidase deficiency

Schuchman, Edward H., He, Xingxuan, Dworski, Shaalee, Zhu, Changzhi, DeAngelis, Victor, Solyom, Alex, Levade, Thierry, Medin, Jeffrey A., Simonaro, Calogera M.

Journal:

Molecular Genetics and Metabolism

Année:

2017

Langue:

english

Fichier:

PDF, 50 KB

english, 2017

21

Lentivector Iterations and Pre-Clinical Scale-Up/Toxicity Testing: Targeting Mobilized CD34 + Cells for Correction of Fabry Disease

Huang, Ju, Khan, Aneal, Au, Bryan C., Barber, Dwayne L., López-Vásquez, Lucía, Prokopishyn, Nicole L., Boutin, Michel, Rothe, Michael, Rip, Jack W., Abaoui, Mona, Nagree, Murtaza S., Dworski, Shaalee,

Journal:

Molecular Therapy — Methods & Clinical Development

Année:

2017

Langue:

english

Fichier:

PDF, 1.78 MB

english, 2017

22

Chronic lung injury and impaired pulmonary function in a mouse model of acid ceramidase deficiency

Yu, Fabian PS, Islam, Diana, Sikora, Jakub, Dworski, Shaalee, Gurka', Jiří, Lopez-Vasquez, Lucia, Liu, Mingyao, Kuebler, Wolfgang Michael, Levade, Thierry, Zhang, Haibo, Medin, Jeffrey A

Journal:

American journal of physiology. Lung cellular and molecular physiology

Année:

2017

Langue:

english

Fichier:

PDF, 4.27 MB

english, 2017

23

Deletion of MCP-1 Impedes Pathogenesis of Acid Ceramidase Deficiency

Yu, Fabian P. S., Dworski, Shaalee, Medin, Jeffrey A.

Journal:

Scientific Reports

Année:

2018

Langue:

english

Fichier:

PDF, 11.06 MB

english, 2018

24

Pathologic Bladder Microenvironment Attenuates Smooth Muscle Differentiation of Skin Derived Precursor Cells: Implications for Tissue Regeneration

Tolg, Cornelia, Ahsan, Alya, Dworski, Shaalee, Kirwan, Tyler, Yu, Jeffery, Aitken, Karen, Bägli, Darius Jehan, Singh, Shree Ram

Journal:

Année:

2013

Langue:

english

Fichier:

PDF, 1021 KB

english, 2013

25

Endogenous levels of 1-O-acylceramides increase upon acidic ceramidase deficiency and decrease due to loss of Dgat1 in a tissue-dependent manner

Bayerle, Aline, Marsching, Christian, Rabionet, Mariona, Dworski, Shaalee, Kamani, Mustafa A., Chitraju, Chandramohan, Gluchowski, Nina L., Gabriel, Katlyn R., Herzer, Silke, Jennemann, Richard, Levad

Journal:

Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids

Année:

2020

Fichier:

PDF, 1.35 MB

2020

26

Transplantation of Skin Precursor-Derived Schwann Cells Yields Better Locomotor Outcomes and Reduces Bladder Pathology in Rats with Chronic Spinal Cord Injury

Assinck, Peggy, Sparling, Joseph S., Dworski, Shaalee, Duncan, Greg J., Wu, Di L., Liu, Jie, Kwon, Brian K., Biernaskie, Jeff, Miller, Freda D., Tetzlaff, Wolfram

Journal:

Stem Cell Reports

Année:

2020

Fichier:

PDF, 7.44 MB

2020