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Mechanism-Based Combination Treatment Dramatically Increases Therapeutic Efficacy In Murine Globoid Cell Leukodystrophy, Jacqueline A. Hawkins-Salsbury, Lauren Shea, Xuntian Jiang, Daniel A. Hunter, A Miguel Guzman, Adarsh S. Reddy, Elizabeth Y. Qin, Yedda Li, Steven J. Gray, Daniel S. Ory, Mark S. Sands Apr 2015

Mechanism-Based Combination Treatment Dramatically Increases Therapeutic Efficacy In Murine Globoid Cell Leukodystrophy, Jacqueline A. Hawkins-Salsbury, Lauren Shea, Xuntian Jiang, Daniel A. Hunter, A Miguel Guzman, Adarsh S. Reddy, Elizabeth Y. Qin, Yedda Li, Steven J. Gray, Daniel S. Ory, Mark S. Sands

Open Access Publications

Globoid cell leukodystrophy (GLD, Krabbe disease) is a lysosomal storage disease (LSD) caused by a deficiency in galactocerebrosidase (GALC) activity. In the absence of GALC activity, the cytotoxic lipid, galactosylsphingosine (psychosine), accumulates in the CNS and peripheral nervous system. Oligodendrocytes and Schwann cells are particularly sensitive to psychosine, thus leading to a demyelinating phenotype. Although hematopoietic stem-cell transplantation provides modest benefit in both presymptomatic children and the murine model (Twitcher), there is no cure for GLD. In addition, GLD has been relatively refractory to virtually every experimental therapy attempted. Here, Twitcher mice were simultaneously treated with CNS-directed gene therapy, substrate …


Adamtsl2 Deletion Results In Bronchial Fibrillin Microfibril Accumulation And Bronchial Epithelial Dysplasia: A Novel Mouse Model Providing Insights On Geleophysic Dysplasia, Dirk Hubmacher, Lauren W. Wang, Robert P. Mecham, Dieter P. Reinhardt, Suneel S. Apte Mar 2015

Adamtsl2 Deletion Results In Bronchial Fibrillin Microfibril Accumulation And Bronchial Epithelial Dysplasia: A Novel Mouse Model Providing Insights On Geleophysic Dysplasia, Dirk Hubmacher, Lauren W. Wang, Robert P. Mecham, Dieter P. Reinhardt, Suneel S. Apte

Open Access Publications

No abstract provided.


Uni-Directional Ciliary Membrane Protein Trafficking By A Cytoplasmic Retrograde Ift Motor And Ciliary Ectosome Shedding, Muqing Cao, Jue Ning, Carmen I. Hernandez-Lara, Olivier Belzile, Qian Wang, Susan K. Dutcher, Yanjie Liu, William J. Snell Feb 2015

Uni-Directional Ciliary Membrane Protein Trafficking By A Cytoplasmic Retrograde Ift Motor And Ciliary Ectosome Shedding, Muqing Cao, Jue Ning, Carmen I. Hernandez-Lara, Olivier Belzile, Qian Wang, Susan K. Dutcher, Yanjie Liu, William J. Snell

Open Access Publications

The role of the primary cilium in key signaling pathways depends on dynamic regulation of ciliary membrane protein composition, yet we know little about the motors or membrane events that regulate ciliary membrane protein trafficking in existing organelles. Recently, we showed that cilium-generated signaling in Chlamydomonas induced rapid, anterograde IFT-independent, cytoplasmic microtubule-dependent redistribution of the membrane polypeptide, SAG1-C65, from the plasma membrane to the periciliary region and the ciliary membrane. Here, we report that the retrograde IFT motor, cytoplasmic dynein 1b, is required in the cytoplasm for this rapid redistribution. Furthermore, signaling-induced trafficking of SAG1-C65 into cilia is unidirectional and …


Highly Sensitive Image-Derived Indices Of Water-Stressed Plants Using Hyperspectral Imaging In Swir And Histogram Analysis, David M. Kim, Hairong Zhang, Haiying Zhou, Tommy Du, Qian Wu, Todd C. Mockler, Mikhail Y. Berezin Jan 2015

Highly Sensitive Image-Derived Indices Of Water-Stressed Plants Using Hyperspectral Imaging In Swir And Histogram Analysis, David M. Kim, Hairong Zhang, Haiying Zhou, Tommy Du, Qian Wu, Todd C. Mockler, Mikhail Y. Berezin

Open Access Publications

No abstract provided.


Cortical Alpha Activity Predicts The Confidence In An Impending Action, Jan Kubanek, N. Jeremy Hill, Lawrence H. Snyder, Gerwin Schalk Jan 2015

Cortical Alpha Activity Predicts The Confidence In An Impending Action, Jan Kubanek, N. Jeremy Hill, Lawrence H. Snyder, Gerwin Schalk

Open Access Publications

No abstract provided.


Cd4+ And Cd8+ T Cells Have Opposing Roles In Breast Cancer Progression And Outcome, Yi Huang, Chunling Ma, Qunyuan Zhang, Jian Ye, Fang Wang, Yanping Zhang, Pamela Hunborg, Mark A. Varvares, Daniel F. Hoft, Eddy C. Hsueh, Guangyong Peng Jan 2015

Cd4+ And Cd8+ T Cells Have Opposing Roles In Breast Cancer Progression And Outcome, Yi Huang, Chunling Ma, Qunyuan Zhang, Jian Ye, Fang Wang, Yanping Zhang, Pamela Hunborg, Mark A. Varvares, Daniel F. Hoft, Eddy C. Hsueh, Guangyong Peng

Open Access Publications

No abstract provided.


Does Diacylglycerol Accumulation In Fatty Liver Disease Cause Hepatic Insulin Resistance?, Brian N. Finck, Angela M. Hall Jan 2015

Does Diacylglycerol Accumulation In Fatty Liver Disease Cause Hepatic Insulin Resistance?, Brian N. Finck, Angela M. Hall

Open Access Publications

No abstract provided.


Imidazolium Salts As Small-Molecule Urinary Bladder Exfoliants In A Murine Model, Patrick O. Wagers, Kristin M. Tiemann, Kerri L. Shelton, William G. Kofron, Matthew J. Panzner, Karen L. Wooley, Wiley J. Youngs, David A. Hunstad Jan 2015

Imidazolium Salts As Small-Molecule Urinary Bladder Exfoliants In A Murine Model, Patrick O. Wagers, Kristin M. Tiemann, Kerri L. Shelton, William G. Kofron, Matthew J. Panzner, Karen L. Wooley, Wiley J. Youngs, David A. Hunstad

Open Access Publications

No abstract provided.


Two Classes Of Regulatory Subunits Coassemble In The Same Bk Channel And Independently Regulate Gating, Vivian Gonzalez-Perez, Xiao-Ming Xia, Christopher J. Lingle Jan 2015

Two Classes Of Regulatory Subunits Coassemble In The Same Bk Channel And Independently Regulate Gating, Vivian Gonzalez-Perez, Xiao-Ming Xia, Christopher J. Lingle

Open Access Publications

No abstract provided.


Antimicrobial Susceptibility Profiles Of Staphylococcus Aureus Isolates Recovered From Humans, Environmental Surfaces, And Companion Animals In Households Of Children With Community-Onset Methicillin-Resistant S. Aureus Infections, John J. Morelli, Patrick G. Hogan, Melanie L. Sullivan, Carol E. Muenks, Jeffrey W. Wang, Ryley M. Thompson, Carey-Ann D. Burnham, Stephanie A. Fritz Jan 2015

Antimicrobial Susceptibility Profiles Of Staphylococcus Aureus Isolates Recovered From Humans, Environmental Surfaces, And Companion Animals In Households Of Children With Community-Onset Methicillin-Resistant S. Aureus Infections, John J. Morelli, Patrick G. Hogan, Melanie L. Sullivan, Carol E. Muenks, Jeffrey W. Wang, Ryley M. Thompson, Carey-Ann D. Burnham, Stephanie A. Fritz

Open Access Publications

No abstract provided.


Trans-Mitochondrial Coordination Of Cristae At Regulated Membrane Junctions, Martin Picard, Meagan J. Mcmanus, György Csordás, Péter Várnai, Gerald W. Dorn Ii, Dewight Williams, György Hajnóczky, Douglas C. Wallace Jan 2015

Trans-Mitochondrial Coordination Of Cristae At Regulated Membrane Junctions, Martin Picard, Meagan J. Mcmanus, György Csordás, Péter Várnai, Gerald W. Dorn Ii, Dewight Williams, György Hajnóczky, Douglas C. Wallace

Open Access Publications

No abstract provided.


Sugarcoated Isolation: Evidence That Social Avoidance Is Linked To Higher Basal Glucose Levels And Higher Consumption Of Glucose, Tsachi Ein-Dor, James A. Coan, Abira Reizer, Elizabeth B. Gross, Dana Dahan, Meredyth A. Wegener, Rafael Carel, Claude R. Cloninger, Ada H. Zohar Jan 2015

Sugarcoated Isolation: Evidence That Social Avoidance Is Linked To Higher Basal Glucose Levels And Higher Consumption Of Glucose, Tsachi Ein-Dor, James A. Coan, Abira Reizer, Elizabeth B. Gross, Dana Dahan, Meredyth A. Wegener, Rafael Carel, Claude R. Cloninger, Ada H. Zohar

Open Access Publications

No abstract provided.


Case Report: Typhoid Fever Complicated By Hemophagocytic Lymphohistiocytosis And Rhabdomyolysis, Lemuel R. Non, Rupa Patel, Amir Esmaeeli, Vladimir Despotovic Jan 2015

Case Report: Typhoid Fever Complicated By Hemophagocytic Lymphohistiocytosis And Rhabdomyolysis, Lemuel R. Non, Rupa Patel, Amir Esmaeeli, Vladimir Despotovic

Open Access Publications

No abstract provided.


Detailed Phenotypic And Molecular Analyses Of Genetically Modified Mice Generated By Crispr-Cas9-Mediated Editing, Bijal A. Parikh, Diana L. Beckman, Swapneel J. Patel, J. Michael White, Wayne M. Yokoyama Jan 2015

Detailed Phenotypic And Molecular Analyses Of Genetically Modified Mice Generated By Crispr-Cas9-Mediated Editing, Bijal A. Parikh, Diana L. Beckman, Swapneel J. Patel, J. Michael White, Wayne M. Yokoyama

Open Access Publications

No abstract provided.


Protein Kinase Cδ Mediates Histamine-Evoked Itch And Responses In Pruriceptors, Manouela V. Valtcheva, Steve Davidson, Chengshui Zhao, Robert W. Gereau Iv Jan 2015

Protein Kinase Cδ Mediates Histamine-Evoked Itch And Responses In Pruriceptors, Manouela V. Valtcheva, Steve Davidson, Chengshui Zhao, Robert W. Gereau Iv

Open Access Publications

No abstract provided.


Small Sized Egfr1 And Her2 Specific Bifunctional Antibody For Targeted Cancer Therapy, Li Ding, Caiping Tian, Song Feng, Guissi Fida, Congying Zhang, Yuxiang Ma, Guanhua Ai, Samuel Achilefu, Yueqing Gu Jan 2015

Small Sized Egfr1 And Her2 Specific Bifunctional Antibody For Targeted Cancer Therapy, Li Ding, Caiping Tian, Song Feng, Guissi Fida, Congying Zhang, Yuxiang Ma, Guanhua Ai, Samuel Achilefu, Yueqing Gu

Open Access Publications

No abstract provided.


Malaria Parasites Produce Volatile Mosquito Attractants, Megan Kelly, Chih-Ying Su, Chad Schaber, Jan R. Crowley, Fong-Fu Hsu, John R. Carlson, Audrey R. Odom Jan 2015

Malaria Parasites Produce Volatile Mosquito Attractants, Megan Kelly, Chih-Ying Su, Chad Schaber, Jan R. Crowley, Fong-Fu Hsu, John R. Carlson, Audrey R. Odom

Open Access Publications

No abstract provided.


Comprehensive Bioimaging With Fluorinated Nanoparticles Using Breathable Liquids, Gary J. Patti, Et Al Jan 2015

Comprehensive Bioimaging With Fluorinated Nanoparticles Using Breathable Liquids, Gary J. Patti, Et Al

Open Access Publications

No abstract provided.


The Lysosomal Enzyme Receptor Protein (Lerp) Is Not Essential, But Is Implicated In Lysosomal Function In Drosophila Melanogaster, Medina Hasanagic, Eline Van Meel, Shan Luan, Rajeev Aurora, Stuart Kornfeld, Joel C. Eissenberg Jan 2015

The Lysosomal Enzyme Receptor Protein (Lerp) Is Not Essential, But Is Implicated In Lysosomal Function In Drosophila Melanogaster, Medina Hasanagic, Eline Van Meel, Shan Luan, Rajeev Aurora, Stuart Kornfeld, Joel C. Eissenberg

Open Access Publications

No abstract provided.


Mitochondrial Biogenesis And Dynamics In The Developing And Diseased Heart, Gerald W. Dorn Ii, Rick B. Vega, Daniel P. Kelly Jan 2015

Mitochondrial Biogenesis And Dynamics In The Developing And Diseased Heart, Gerald W. Dorn Ii, Rick B. Vega, Daniel P. Kelly

Open Access Publications

No abstract provided.


The Role Of Dopamine And Dopaminergic Pathways In Dystonia: Insights From Neuroimaging, Morvarid Karimi, Joel S Perlmutter Jan 2015

The Role Of Dopamine And Dopaminergic Pathways In Dystonia: Insights From Neuroimaging, Morvarid Karimi, Joel S Perlmutter

Open Access Publications

BACKGROUND: Dystonia constitutes a heterogeneous group of movement abnormalities, characterized by sustained or intermittent muscle contractions causing abnormal postures. Overwhelming data suggest involvement of basal ganglia and dopaminergic pathways in dystonia. In this review, we critically evaluate recent neuroimaging studies that investigate dopamine receptors, endogenous dopamine release, morphology of striatum, and structural or functional connectivity in cortico-basal ganglia-thalamo-cortical and related cerebellar circuits in dystonia.

METHOD: A PubMed search was conducted in August 2014.

RESULTS: Positron emission tomography (PET) imaging offers strong evidence for altered D2/D3 receptor binding and dopaminergic release in many forms of idiopathic dystonia. Functional magnetic resonance imaging …


Sumoylation Is Developmentally Regulated And Required For Cell Pairing During Conjugation In Tetrahymena Thermophila, Amjad M. Nasir, Qianyi Yang, Douglas L. Chalker, James D. Forney Jan 2015

Sumoylation Is Developmentally Regulated And Required For Cell Pairing During Conjugation In Tetrahymena Thermophila, Amjad M. Nasir, Qianyi Yang, Douglas L. Chalker, James D. Forney

Open Access Publications

No abstract provided.


Species-Specific Transmission Of Novel Picornaviruses In Lemurs, Efrem S. Lim, Sharon L. Deem, Ingrid J. Porton, Song Cao, David Wang Jan 2015

Species-Specific Transmission Of Novel Picornaviruses In Lemurs, Efrem S. Lim, Sharon L. Deem, Ingrid J. Porton, Song Cao, David Wang

Open Access Publications

No abstract provided.


Comprehensive Functional Characterization Of Murine Infantile Batten Disease Including Parkinson-Like Behavior And Dopaminergic Markers, Joshua T. Dearborn, Steven K. Harmon, Stephen C. Fowler, Karen L. O'Malley, George T. Taylor, Mark S. Sands, David F. Wozniak Jan 2015

Comprehensive Functional Characterization Of Murine Infantile Batten Disease Including Parkinson-Like Behavior And Dopaminergic Markers, Joshua T. Dearborn, Steven K. Harmon, Stephen C. Fowler, Karen L. O'Malley, George T. Taylor, Mark S. Sands, David F. Wozniak

Open Access Publications

No abstract provided.


On The Existence Of A Generalized Non-Specific Task-Dependent Network, Kenneth Hugdahl, Marcus E. Raichle, Anish Mitra, Karsten Specht Jan 2015

On The Existence Of A Generalized Non-Specific Task-Dependent Network, Kenneth Hugdahl, Marcus E. Raichle, Anish Mitra, Karsten Specht

Open Access Publications

No abstract provided.


Role Of The Clathrin Adaptor Picalm In Normal Hematopoiesis And Polycythemia Vera Pathophysiology, Yuichi Ishikawa, Manami Maeda, Mithun Pasham, Francois Aguet, Silvia K. Tacheva-Grigorova, Takeshi Masuda, Hai Yi, Sung-Uk Lee, Jian Xu, Julie Teruya-Feldstein, Maria Ericsson, Ann Mullally, John Heuser, Tom Kirchhausen, Takahiro Maeda Jan 2015

Role Of The Clathrin Adaptor Picalm In Normal Hematopoiesis And Polycythemia Vera Pathophysiology, Yuichi Ishikawa, Manami Maeda, Mithun Pasham, Francois Aguet, Silvia K. Tacheva-Grigorova, Takeshi Masuda, Hai Yi, Sung-Uk Lee, Jian Xu, Julie Teruya-Feldstein, Maria Ericsson, Ann Mullally, John Heuser, Tom Kirchhausen, Takahiro Maeda

Open Access Publications

No abstract provided.


Matching Behavior As A Tradeoff Between Reward Maximization And Demands On Neural Computation, Jan Kubanek, Lawrence H. Snyder Jan 2015

Matching Behavior As A Tradeoff Between Reward Maximization And Demands On Neural Computation, Jan Kubanek, Lawrence H. Snyder

Open Access Publications

No abstract provided.


Systemic Ep4 Inhibition Increases Adhesion Formation In A Murine Model Of Flexor Tendon Repair, Michael B. Geary, Caitlin A. Orner, Fatima Bawany, Hani A. Awad, Warren C. Hammert, Regis J. O'Keefe, Alayna E. Loiselle Jan 2015

Systemic Ep4 Inhibition Increases Adhesion Formation In A Murine Model Of Flexor Tendon Repair, Michael B. Geary, Caitlin A. Orner, Fatima Bawany, Hani A. Awad, Warren C. Hammert, Regis J. O'Keefe, Alayna E. Loiselle

Open Access Publications

No abstract provided.


The Amazing Brain, Krikor Dikranian Jan 2015

The Amazing Brain, Krikor Dikranian

Open Access Publications

No abstract provided.


Interventions To Slow Aging In Humans: Are We Ready?, Samuel Klein, Et Al Jan 2015

Interventions To Slow Aging In Humans: Are We Ready?, Samuel Klein, Et Al

Open Access Publications

No abstract provided.