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1

Converging pathways in neurodegeneration, from genetics to mechanisms

Gan, Li, Cookson, Mark R., Petrucelli, Leonard, La Spada, Albert R.

Journal:

Nature Neuroscience

Year:

2018

Language:

english

File:

PDF, 2.15 MB

english, 2018

2

CTCF Regulates Ataxin-7 Expression through Promotion of a Convergently Transcribed, Antisense Noncoding RNA

Bryce L. Sopher, Paula D. Ladd, Victor V. Pineda, Randell T. Libby, Susan M. Sunkin, James B. Hurley, Cortlandt P. Thienes, Terry Gaasterland, Galina N. Filippova, Albert R. La Spada

Journal:

Year:

2011

Language:

english

File:

PDF, 1.26 MB

english, 2011

3

Androgen Receptor Function in Motor Neuron Survival and Degeneration

Cary, Gregory A., La Spada, Albert R.

Journal:

Physical Medicine and Rehabilitation Clinics of North America

Year:

2008

Language:

english

File:

PDF, 295 KB

english, 2008

4

Targeting protein aggregation in neurodegeneration - lessons from polyglutamine disorders

Weydt, Patrick, La Spada, Albert R

Journal:

Expert Opinion on Therapeutic Targets

Year:

2006

Language:

english

File:

PDF, 166 KB

english, 2006

5

Familial Dementia With Lewy Bodies

Tsuang, Debby W., Dalan, Aaron M., Eugenio, Charisma J., Poorkaj, Parvonah, Limprasert, Pornprot, La Spada, Albert R., Steinbart, Ellen J., Bird, Thomas D., Leverenz, James B.

Journal:

Archives of Neurology

Year:

2002

Language:

english

File:

PDF, 223 KB

english, 2002

6

4E-BP1 protects neurons from misfolded protein stress and Parkinsonâs disease toxicity by inducing the mitochondrial unfolded protein response

Dastidar, Somasish Ghosh, Pham, Michael T., Mitchell, Matthew B., Yeom, Steven G., Jordan, Sarah, Chang, Angela, Sopher, Bryce L., La Spada, Albert R.

Journal:

Journal of Neuroscience

Year:

2020

File:

PDF, 4.84 MB

2020

7

Repeat expansion disease: progress and puzzles in disease pathogenesis

La Spada, Albert R., Taylor, J. Paul

Journal:

Nature Reviews Genetics

Year:

2010

Language:

english

File:

PDF, 484 KB

english, 2010

8

A SCA7 CAG/CTG repeat expansion is stable in Drosophila melanogaster despite modulation of genomic context and gene dosage

Stephen M. Jackson, Alex J. Whitworth, Jessica C. Greene, Randell T. Libby, Sandy L. Baccam, Leo J. Pallanck, Albert R. La Spada

Journal:

Year:

2005

Language:

english

File:

PDF, 229 KB

english, 2005

9

Polyglutamines Stop Traffic: Axonal Transport as a Common Target in Neurodegenerative Diseases

Mel B Feany, Albert R La Spada

Journal:

Year:

2003

Language:

english

File:

PDF, 41 KB

english, 2003

10

Astroglial-targeted expression of the fragile X CGG repeat premutation in mice yields RAN translation, motor deficits and possible evidence for cell-to-cell propagation of FXTAS pathology

Wenzel, H. Jürgen, Murray, Karl D., Haify, Saif N., Hunsaker, Michael R., Schwartzer, Jared J., Kim, Kyoungmi, La Spada, Albert R., Sopher, Bryce L., Hagerman, Paul J., Raske, Christopher, Severijnen,

Journal:

Acta Neuropathologica Communications

Year:

2019

Language:

english

File:

PDF, 14.16 MB

english, 2019

11

Low-Cost Gait Analysis for Behavioral Phenotyping of Mouse Models of Neuromuscular Disease

Wertman, Virginia, Gromova, Anastasia, La Spada, Albert R., Cortes, Constanza J.

Journal:

Journal of Visualized Experiments

Year:

2019

Language:

english

File:

PDF, 324 KB

english, 2019

12

Points to consider: is there evidence to support BRCA1/2 and other inherited breast cancer genetic testing for all breast cancer patients? A statement of the American College of Medical Genetics and Genomics (ACMG)

Pal, Tuya, Agnese, Doreen, Daly, Mary, La Spada, Albert, Litton, Jennifer, Wick, Myra, Klugman, Susan, Esplin, Edward D., Jarvik, Gail P.

Journal:

Genetics in Medicine

Year:

2019

File:

PDF, 259 KB

2019

13

Déjà vu with a twist: transglutaminases in bioenergetics and transcriptional dysfunction in Huntington's disease

Parsa Kazemi-Esfarjani, Albert R. La Spada

Journal:

EMBO Molecular Medicine

Year:

2010

Language:

english

File:

PDF, 123 KB

english, 2010

14

Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy

Spada, Albert R. La, Wilson, Elizabeth M, Lubahn, Dennis B, Harding, A. E, Fischbeck, Kenneth H

Journal:

Year:

1991

File:

PDF, 362 KB

1991

15

Efficient recombination-based methods for bacterial artificial chromosome fusion and mutagenesis

Bryce L. Sopher, Albert R. La Spada

Journal:

Year:

2006

Language:

english

File:

PDF, 337 KB

english, 2006

16

Small-bowel capsule endoscopy and device-assisted enteroscopy for diagnosis and treatment of small-bowel disorders: European Society of Gastrointestinal Endoscopy (ESGE) Clinical Guideline

Pennazio, Marco, Spada, Cristiano, Eliakim, Rami, Keuchel, Martin, May, Andrea, Mulder, Chris, Rondonotti, Emanuele, Adler, Samuel, Albert, Joerg, Baltes, Peter, Barbaro, Federico, Cellier, Christophe

Journal:

Year:

2015

Language:

english

File:

PDF, 518 KB

english, 2015

17

Disease-modifying effect of adiponectin in models of alpha-synucleinopathies in vitro and in vivo

Waragai, Masaaki, Sekiyama, Kazunari, Takamatsu, Yoshiki, Fujita, Masayo, Rockenstien, Edward, Uemura, Kenichi, Iwai, Naomichi, Inoue, Satoshi, La Spada, Albert R., Masliah, Eliezer, Hashimoto, Makoto

Journal:

Alzheimer's & Dementia

Year:

2015

Language:

english

File:

PDF, 477 KB

english, 2015

18

Trehalase polymorphism inDrosophila melanogaster

Ronald S. Burton, Albert Spada

Journal:

Biochemical Genetics

Year:

1986

Language:

english

File:

PDF, 263 KB

english, 1986

19

Meiotic stability and genotype – phenotype correlation of the trinucleotide repeat in X–linked spinal and bulbar muscular atrophy

La Spada, Albert R., Roling, Daniel B., Harding, Anita E., Warner, Carolyn L., Spiegel, Roland, Hausmanowa-Petrusewicz, Irena, Yee, Woon-Chee, Fischbeck, Kenneth H.

Journal:

Nature Genetics

Year:

1992

File:

PDF, 444 KB

1992

20

Autophagy activation and enhanced mitophagy characterize the Purkinje cells ofpcdmice prior to neuronal death

Lisa Chakrabarti, Jeremiah Eng, Nishi Ivanov, Gwenn A Garden, Albert R La Spada

Journal:

Molecular Brain

Year:

2009

Language:

english

File:

PDF, 2.76 MB

english, 2009

21

Androgen Receptor YAC Transgenic Mice Recapitulate SBMA Motor Neuronopathy and Implicate VEGF164 in the Motor Neuron Degeneration

Bryce L Sopher, Patrick S Thomas Jr., Michelle A LaFevre-Bernt, Ida E Holm, Scott A Wilke, Carol B Ware, Lee-Way Jin, Randell T Libby, Lisa M Ellerby, Albert R La Spada

Journal:

Year:

2004

Language:

english

File:

PDF, 569 KB

english, 2004

22

The expanding world of stem cell modeling of Huntington's disease: creating tools with a promising future

Jacqueline M Ward,Albert R La Spada

Journal:

Genome Medicine

Year:

2012

Language:

english

File:

PDF, 160 KB

english, 2012

23

Transcriptional regulation of core autophagy and lysosomal genes by the androgen receptor promotes prostate cancer progression

Blessing, Alicia M., Rajapakshe, Kimal, Reddy Bollu, Lakshmi, Shi, Yan, White, Mark A., Pham, Alexander H., Lin, Chenchu, Jonsson, Philip, Cortes, Constanza J., Cheung, Edwin, La Spada, Albert R., Bas

Journal:

Year:

2017

Language:

english

File:

PDF, 2.05 MB

english, 2017

24

Memantine strikes the perfect balance

La Spada, Albert R

Journal:

Nature Medicine

Year:

2009

Language:

english

File:

PDF, 2.16 MB

english, 2009

25

Something wicked this way comes: huntingtin

La Spada, Albert R

Journal:

Nature Neuroscience

Year:

2014

Language:

english

File:

PDF, 684 KB

english, 2014

26

A Simple Evaluation Tool (ET-CET) Indicates Increase of Diagnostic Skills From Small Bowel Capsule Endoscopy Training Courses

Albert, J.G., Humbla, O., McAlindon, M.E., Davison, C., Seitz, U., Fraser, C., Hagenmüller, F., Noetzel, E., Spada, C., Riccioni, M.E., Barnert, J., Filmann, N., Keuchel, M.

Journal:

Medicine (Medicine Publishing Company Ltd)

Year:

2015

Language:

english

File:

PDF, 545 KB

english, 2015

27

A Becn1 mutation mediates hyperactive autophagic sequestration of amyloid oligomers and improved cognition in Alzheimer's disease

Rocchi, Altea, Yamamoto, Soh, Ting, Tabitha, Fan, Yuying, Sadleir, Katherine, Wang, Yigang, Zhang, Weiran, Huang, Sui, Levine, Beth, Vassar, Robert, He, Congcong, La Spada, Albert R.

Journal:

Year:

2017

Language:

english

File:

PDF, 11.35 MB

english, 2017

28

MITOCHONDRIAL DYSFUNCTION IN IPSC-DERIVED NEURONS OF SUBJECTS WITH CHRONIC MOUNTAIN SICKNESS

Zhao, Helen, Perkins, Guy A., Yao, Hang, Callacondo, David, Appenzeller, Otto, Ellisman, Mark, La Spada, Albert R., Haddad, Gabriel G.

Journal:

Journal of Applied Physiology

Year:

2017

Language:

english

File:

PDF, 734 KB

english, 2017

29

PPARGC1A/PGC-1α, TFEB, and enhanced proteostasis in Huntington disease: Defining regulatory linkages between energy production and protein–organelle quality control

La Spada, Albert R.

Journal:

Year:

2012

Language:

english

File:

PDF, 682 KB

english, 2012

30

Glutamine Repeats and Neurodegenerative Diseases: Molecular Aspects

Albert R. La Spada

Journal:

The American Journal of Human Genetics

Year:

2002

Language:

english

File:

PDF, 35 KB

english, 2002

31

Visceral Neuropathy and Intestinal Pseudo-Obstruction in a Murine Model of a Nuclear Inclusion Disease

Christine M. Clarke, Cara Plata, Bonnie Cole, Karen Tsuchiya, Albert R. La Spada, Raj P. Kapur

Journal:

Gastroenterology

Year:

2007

Language:

english

File:

PDF, 1.81 MB

english, 2007

32

Development of selective nutrient deprivation as a system to study autophagy induction and regulation in neurons

Young, Jessica, La Spada, Albert R.

Journal:

Year:

2009

Language:

english

File:

PDF, 367 KB

english, 2009

33

WITHDRAWN: Visceral neuropathy and intestinal pseudo-obstruction in a murine model of a nuclear inclusion disease

Clarke, Christine M., Plata, Cara, Cole, Bonnie, Tsuchiya, Karen, La Spada, Albert R., Kapur, Raj P.

Journal:

Gastroenterology

Year:

2007

File:

PDF, 12 KB

2007

34

Tight expression regulation of senataxin, linked to motor neuron disease and ataxia, is required to avert cell-cycle block and nucleolus disassembly

Bennett, Craig L., Sopher, Bryce L., La Spada, Albert R.

Journal:

Year:

2020

File:

PDF, 1.37 MB

2020

35

S-Nitrosylation of Dynamin-Related Protein 1 Mediates Mutant Huntingtin-Induced Mitochondrial Fragmentation and Neuronal Injury in Huntington's Disease

Haun, Florian, Nakamura, Tomohiro, Shiu, Alicia D., Cho, Dong-Hyung, Tsunemi, Taiji, Holland, Emily A., La Spada, Albert R., Lipton, Stuart A.

Journal:

Antioxidants & Redox Signaling

Year:

2013

Language:

english

File:

PDF, 1.26 MB

english, 2013

36

Fatal Hemorrhage in a Patient with an Acquired Inhibitor of Human Thrombin

Spada, Albert R. La, Skålhegg, Bjørn S., Henderson, Ruth, Schmer, Gottfried, Pierce, Robert, Chandler, Wayne

Journal:

Boston Medical and Surgical Journal

Year:

1995

Language:

english

File:

PDF, 104 KB

english, 1995

37

β-synuclein in the pathogenesis of Parkinson’s disease and related α-synucleinopathies: emerging roles and new directions

Hashimoto, Makoto, La Spada, Albert R

Journal:

Future Neurology

Year:

2012

Language:

english

File:

PDF, 525 KB

english, 2012

38

MAP4K3 mediates amino acid-dependent regulation of autophagy via phosphorylation of TFEB

Hsu, Cynthia L., Lee, Elian X., Gordon, Kara L., Paz, Edwin A., Shen, Wen-Chuan, Ohnishi, Kohta, Meisenhelder, Jill, Hunter, Tony, La Spada, Albert R.

Journal:

Nature Communications

Year:

2018

Language:

english

File:

PDF, 3.59 MB

english, 2018

39

CCP1 promotes mitochondrial fusion and motility to prevent Purkinje cell neuron loss in pcd mice

Gilmore-Hall, Stephen, Kuo, Jennifer, Ward, Jacqueline M., Zahra, Rabaab, Morrison, Richard S., Perkins, Guy, La Spada, Albert R.

Journal:

The Journal of Cell Biology

Year:

2018

Language:

english

File:

PDF, 2.73 MB

english, 2018

40

Polyglutamine-Expanded Ataxin-7 Antagonizes CRX Function and Induces Cone-Rod Dystrophy in a Mouse Model of SCA7

Albert R. La Spada, Ying-Hui Fu, Bryce L. Sopher, Randell T. Libby, Xuejiao Wang, Lili Y. Li, David D. Einum, Jing Huang, Daniel E. Possin, Annette C. Smith, Refugio A. Martinez, Kari L. Koszdin, Pipe

Journal:

Year:

2001

Language:

english

File:

PDF, 947 KB

english, 2001

41

FUS is not dysregulated by the spinal bulbar muscular atrophy androgen receptor polyglutamine repeat expansion

Fratta, Pietro, Malik, Bilal, Gray, Anna, La Spada, Albert R., Hanna, Michael G., Fisher, Elizabeth M.C., Greensmith, Linda

Journal:

Neurobiology of Aging

Year:

2013

Language:

english

File:

PDF, 456 KB

english, 2013

42

Dynamic mutations on the move in Banff

Spada, Albert R La, Richards, Robert I, Wieringa, Bé

Journal:

Nature Genetics

Year:

2004

Language:

english

File:

PDF, 298 KB

english, 2004

43

The Power of the Dark Side: Huntington’s Disease Protein and p53 Form a Deadly Alliance

Albert R. La Spada, Richard S. Morrison

Journal:

Year:

2005

Language:

english

File:

PDF, 74 KB

english, 2005

44

Peripheral Androgen Receptor Gene Suppression Rescues Disease in Mouse Models of Spinal and Bulbar Muscular Atrophy

Lieberman, Andrew P., Yu, Zhigang, Murray, Sue, Peralta, Raechel, Low, Audrey, Guo, Shuling, Yu, Xing Xian, Cortes, Constanza J., Bennett, C. Frank, Monia, Brett P., La Spada, Albert R., Hung, Gene

Journal:

Year:

2014

Language:

english

File:

PDF, 2.57 MB

english, 2014

45

Therapy development in Huntington disease: From current strategies to emerging opportunities

Dickey, Audrey S., La Spada, Albert R.

Journal:

American Journal of Medical Genetics Part A

Year:

2017

Language:

english

File:

PDF, 1.67 MB

english, 2017

46

Familial Dementia with Lewy Bodies with an Atypical Clinical Presentation

Bonner, Lauren T., Tsuang, Debby W., Cherrier, Monique M., Eugenio, Charisma J., Du, Jennifer Q., Steinbart, Ellen J., Limprasert, Pornprot, La Spada, Albert R., Seltzer, Benjamin, Bird, Thomas D., Le

Journal:

Journal of Geriatric Psychiatry and Neurology

Year:

2003

Language:

english

File:

PDF, 198 KB

english, 2003

47

Low-Cost Gait Analysis for Behavioral Phenotyping of Mouse Models of Neuromuscular Disease

Wertman, Virginia, Gromova, Anastasia, La Spada, Albert R., Cortes, Constanza J.

Journal:

Journal of Visualized Experiments

Year:

2019

File:

PDF, 324 KB

2019

48

Inherited Neurodegenerative Disorders Caused by CAG/Polyglutamine Tract Expansions: Symposium Introduction

Albert R. La Spada, Arthur W. Clark

Journal:

Brain Pathology

Year:

1997

Language:

english

File:

PDF, 279 KB

english, 1997

49

Getting a handle on Huntington's disease: silencing neurodegeneration

La Spada, Albert R

Journal:

Nature Medicine

Year:

2009

Language:

english

File:

PDF, 91 KB

english, 2009

50

An Antisense CAG Repeat Transcript at JPH3 Locus Mediates Expanded Polyglutamine Protein Toxicity in Huntington's Disease-like 2 Mice

Brian Wilburn, Dobrila D. Rudnicki, Jing Zhao, Tara Murphy Weitz, Yin Cheng, Xiaofeng Gu, Erin Greiner, Chang Sin Park, Nan Wang, Bryce L. Sopher, Albert R. La Spada, Alex Osmand, Russell L. Margolis,

Journal:

Year:

2011

Language:

english

File:

PDF, 1.88 MB

english, 2011

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