Search Results - "Tatyana V. Taksir"

Neutral Lipid Cacostasis Contributes to Disease Pathogenesis in Amyotrophic Lateral Sclerosis by Dodge, James C, Jensen, Elizabeth H, Yu, Jinlong, Sardi, S Pablo, Bialas, Allison R, Taksir, Tatyana V, Bangari, Dinesh S, Shihabuddin, Lamya S

“…Amyotrophic lateral sclerosis (ALS) is a fatal neuromuscular disease characterized by motor neuron (MN) death. Lipid dysregulation manifests during disease;…”
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Metabolic signatures of amyotrophic lateral sclerosis reveal insights into disease pathogenesis by Dodge, James C., Treleaven, Christopher M., Fidler, Jonathan A., Tamsett, Thomas J., Bao, Channa, Searles, Michelle, Taksir, Tatyana V., Misra, Kuma, Sidman, Richard L., Cheng, Seng H., Shihabuddin, Lamya S.

“…Metabolic dysfunction is an important modulator of disease course in amyotrophic lateral sclerosis (ALS). We report here that a familial mouse model…”
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AAV4-mediated Expression of IGF-1 and VEGF Within Cellular Components of the Ventricular System Improves Survival Outcome in Familial ALS Mice by Dodge, James C, Treleaven, Christopher M, Fidler, Jonathan A, Hester, Mark, Haidet, Amanda, Handy, Chalonda, Rao, Meghan, Eagle, Amy, Matthews, Jennifer C, Taksir, Tatyana V, Cheng, Seng H, Shihabuddin, Lamya S, Kaspar, Brian K

“…Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by motor neuron cell death in the cortex, brainstem, and spinal cord…”
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Glucosylceramide synthase inhibition reduces ganglioside GM3 accumulation, alleviates amyloid neuropathology, and stabilizes remote contextual memory in a mouse model of Alzheimer's disease by Dodge, James C, Tamsett, Thomas J, Treleaven, Christopher M, Taksir, Tatyana V, Piepenhagen, Peter, Sardi, S Pablo, Cheng, Seng H, Shihabuddin, Lamya S

“…Gangliosides are highly enriched in the brain and are critical for its normal development and function. However, in some rare neurometabolic diseases, a…”
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Delivery of AAV-IGF-1 to the CNS Extends Survival in ALS Mice Through Modification of Aberrant Glial Cell Activity by Dodge, James C, Haidet, Amanda M, Yang, Wendy, Passini, Marco A, Hester, Mark, Clarke, Jennifer, Roskelley, Eric M, Treleaven, Christopher M, Rizo, Liza, Martin, Heather, Kim, Soo H, Kaspar, Rita, Taksir, Tatyana V, Griffiths, Denise A, Cheng, Seng H, Shihabuddin, Lamya S, Kaspar, Brian K

“…Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease of the motor system. Recent work in rodent models of ALS has shown that insulin-like…”
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Combination Brain and Systemic Injections of AAV Provide Maximal Functional and Survival Benefits in the Niemann-Pick Mouse by Passini, Marco A., Bu, Jie, Fidler, Jonathan A., Ziegler, Robin J., Foley, Joseph W., Dodge, James C., Yang, Wendy W., Clarke, Jennifer, Taksir, Tatyana V., Griffiths, Denise A., Zhao, Michael A., O'Riordan, Catherine R., Schuchman, Edward H., Shihabuddin, Lamya S., Cheng, Seng H.

“…Niemann-Pick disease (NPD) is caused by the loss of acid sphingomyelinase (ASM) activity, which results in widespread accumulation of undegraded lipids in…”
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Gene Transfer of Human Acid Sphingomyelinase Corrects Neuropathology and Motor Deficits in a Mouse Model of Niemann-Pick Type A Disease by James C. Dodge, Jennifer Clarke, Antonius Song, Jie Bu, Yang, Wendy, Tatyana V. Taksir, Denise Griffiths, Michael A. Zhao, Schuchman, Edward H., Cheng, Seng H., Catherine R. O'Riordan, Shihabuddin, Lamya S., Marco A. Passini, Stewart, Gregory R.

“…Niemann-Pick type A disease is a lysosomal storage disorder caused by a deficiency in acid sphingomyelinase (ASM) activity. Previously we showed that storage…”
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Optimization of a Histopathological Biomarker for Sphingomyelin Accumulation in Acid Sphingomyelinase Deficiency by Taksir, Tatyana V., Johnson, Jennifer, Maloney, Colleen L., Yandl, Emily, Griffiths, Denise, Thurberg, Beth L., Ryan, Susan

“…Niemann-Pick disease (types A and B), or acid sphingomyelinase deficiency, is an inherited deficiency of acid sphingomyelinase, resulting in intralysosomal…”
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Comparative analysis of acid sphingomyelinase distribution in the CNS of rats and mice following intracerebroventricular delivery by Treleaven, Christopher M, Tamsett, Thomas, Fidler, Jonathan A, Taksir, Tatyana V, Cheng, Seng H, Shihabuddin, Lamya S, Dodge, James C

“…Niemann-Pick A (NPA) disease is a lysosomal storage disorder (LSD) caused by a deficiency in acid sphingomyelinase (ASM) activity. Previously, we reported that…”
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AAV Vector-Mediated Correction of Brain Pathology in a Mouse Model of Niemann–Pick A Disease by Passini, Marco A., Macauley, Shannon L., Huff, Michael R., Taksir, Tatyana V., Bu, Jie, Wu, I-Huan, Piepenhagen, Peter A., Dodge, James C., Shihabuddin, Lamya S., O'Riordan, Catherine R., Schuchman, Edward H., Stewart, Gregory R.

“…Niemann–Pick A disease (NPA) is a fatal lysosomal storage disorder caused by a deficiency in acid sphingomyelinase (ASM) activity. The lack of functional ASM…”
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Intracerebroventricular infusion of acid sphingomyelinase corrects CNS manifestations in a mouse model of Niemann–Pick A disease by Dodge, James C., Clarke, Jennifer, Treleaven, Christopher M., Taksir, Tatyana V., Griffiths, Denise A., Yang, Wendy, Fidler, Jonathan A., Passini, Marco A., Karey, Kenneth P., Schuchman, Edward H., Cheng, Seng H., Shihabuddin, Lamya S.

“…Niemann–Pick A (NPA) disease is a lysosomal storage disorder (LSD) caused by a deficiency in acid sphingomyelinase (ASM) activity. Previously, we showed that…”
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Relationship between neuropathology and disease progression in the SOD1(G93A) ALS mouse by Yang, Wendy W, Sidman, Richard L, Taksir, Tatyana V, Treleaven, Christopher M, Fidler, Jonathan A, Cheng, Seng H, Dodge, James C, Shihabuddin, Lamya S

“…Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the progressive loss of upper and lower motor neurons. However,…”
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Relationship between neuropathology and disease progression in the SOD1G93A ALS mouse by YANG, Wendy W, SIDMAN, Richard L, TAKSIR, Tatyana V, TRELEAVEN, Christopher M, FIDLER, Jonathan A, CHENG, Seng H, DODGE, James C, SHIHABUDDIN, Lamya S

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Ability of adeno-associated virus serotype 8-mediated hepatic expression of acid alpha-glucosidase to correct the biochemical and motor function deficits of presymptomatic and symptomatic Pompe mice by Ziegler, Robin J, Bercury, Scott D, Fidler, Jonathan, Zhao, Michael A, Foley, Joseph, Taksir, Tatyana V, Ryan, Susan, Hodges, Bradley L, Scheule, Ronald K, Shihabuddin, Lamya S, Cheng, Seng H

“…The availability of a murine model of Pompe disease has enabled an evaluation of the relative merits of various therapeutic paradigms, including gene therapy…”
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Optimized Preservation of CNS Morphology for the Identification of Glycogen in the Pompe Mouse Model by Taksir, Tatyana V, Griffiths, Denise, Johnson, Jennifer, Ryan, Susan, Shihabuddin, Lamya S, Thurberg, Beth L

“…Pompe disease (glycogenosis type II) is a rare lysosomal disorder caused by a mutational deficiency of acid α-glucosidase (GAA). This deficiency leads to…”
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Intraparenchymal injections of acid sphingomyelinase results in regional correction of lysosomal storage pathology in the Niemann–Pick A mouse by Yang, Wendy W., Dodge, James C., Passini, Marco A., Taksir, Tatyana V., Griffiths, Denise, Schuchman, Edward H., Cheng, Seng H., Shihabuddin, Lamya S.

“…Niemann–Pick A disease (NPD-A) is caused by a deficiency of acid sphingomyelinase (ASM) leading to the intracellular accumulation of sphingomyelin and…”
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2. Identification of Different Modes of Viral Transport in the Non-Human Primate Brain after Convection-Enhanced Delivery of AAV Serotype Vectors by Passini, Marco A., Bu, Jie, Dodge, James C., Shihabuddin, Lamya S., Taksir, Tatyana V., Griffiths, Denise A., Bringas, John, Hadaczek, Piotr, Avramut, Mihaela, Cheng, Seng H., Bankiewicz, Krystof

“…Recombinant adeno-associated virus serotypes-1 and -2 encoding human acid sphingomyelinase were administered into multiple structures of the cynomolgus monkey…”
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1062. Intracerebellar Injection of AAV-IGF-1 Improves Motor Function and Extends Survival in a Mouse Model of Amyotrophic Lateral Sclerosis by Dodge, James C., Passini, Marco A., Clarke, Jennifer, Grissett, Liza, Kim, Soo Hyun, Wen, Rita, Taksir, Tatyana V., Griffiths, Denise A., Cheng, Seng H., Kaspar, Brian K., Shihabuddin, Lamya S.

“…Amytrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that is characterized by a selective loss of motor neurons in the motor cortex, brain…”
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Mouse neural progenitor cells differentiate into oligodendrocytes in the brain of a knockout mouse model of Canavan disease by Surendran, Sankar, Shihabuddin, Lamya S., Clarke, Jennifer, Taksir, Tatyana V., Stewart, Gregory R., Parsons, Geoffrey, Yang, Wendy, Tyring, Stephen K., Michals-Matalon, Kimberlee, Matalon, Reuben

“…Canavan disease (CD) is an autosomal recessive disorder that leads to spongy degeneration in the white matter of the brain. Aspartoacylase (ASPA) synthesizing…”
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Relationship between neuropathology and disease progression in the SOD1 super(G93A) ALS mouse by Yang, Wendy W, Sidman, Richard L, Taksir, Tatyana V, Treleaven, Christopher M, Fidler, Jonathan A, Cheng, Seng H, Dodge, James C, Shihabuddin, Lamya S

“…Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the progressive loss of upper and lower motor neurons. However,…”
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