Search Results - "SCHUCHMAN, Edward H"

Acid sphingomyelinase, cell membranes and human disease: Lessons from Niemann–Pick disease by Schuchman, Edward H.

“…Acid sphingomyelinase (ASM) plays an important role in normal membrane turnover through the hydrolysis of sphingomyelin, and is one of the key enzymes…”
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Deregulation of sphingolipid metabolism in Alzheimer's disease by He, Xingxuan, Huang, Yu, Li, Bin, Gong, Cheng-Xin, Schuchman, Edward H

“…Abstract Abnormal sphingolipid metabolism has been previously reported in Alzheimer's disease (AD). To extend these findings, several sphingolipids and…”
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unexpected role of acid sphingomyelinase in cell death and the pathophysiology of common diseases by Smith, Eric L, Schuchman, Edward H

“…Acid sphingomyelinase (ASM; E.C. 3.1.4.12) is best known for its involvement in the lysosomal storage disorder Niemann-Pick disease (NPD). Through studies that…”
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Acid sphingomyelinase modulates the autophagic process by controlling lysosomal biogenesis in Alzheimer's disease by Lee, Jong Kil, Jin, Hee Kyung, Park, Min Hee, Kim, Bo-ra, Lee, Phil Hyu, Nakauchi, Hiromitsu, Carter, Janet E, He, Xingxuan, Schuchman, Edward H, Bae, Jae-sung

“…In Alzheimer's disease (AD), abnormal sphingolipid metabolism has been reported, although the pathogenic consequences of these changes have not been fully…”
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Control of Endothelial Targeting and Intracellular Delivery of Therapeutic Enzymes by Modulating the Size and Shape of ICAM-1-targeted Carriers by Muro, Silvia, Garnacho, Carmen, Champion, Julie A, Leferovich, John, Gajewski, Christine, Schuchman, Edward H, Mitragotri, Samir, Muzykantov, Vladimir R

“…Endocytosis in endothelial cells (ECs) is important for many biomedical applications, including drug delivery by nano- and microscale carriers. However, little…”
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Types A and B Niemann-Pick disease by Schuchman, Edward H., PhD, Wasserstein, Melissa P., MD

“…Two distinct metabolic abnormalities are encompassed under the eponym Niemann-Pick disease (NPD). The first is due to the deficient activity of the enzyme acid…”
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Identification of a Novel Acid Sphingomyelinase Activity Associated with Recombinant Human Acid Ceramidase by He, Xingxuan, Schuchman, Edward H

“…Acid ceramidase (AC) is a lysosomal enzyme required to hydrolyze ceramide to sphingosine by the removal of the fatty acid moiety. An inherited deficiency in…”
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Intracerebral Transplantation of Bone Marrow‐Derived Mesenchymal Stem Cells Reduces Amyloid‐Beta Deposition and Rescues Memory Deficits in Alzheimer's Disease Mice by Modulation of Immune Responses by Lee, Jong Kil, Jin, Hee Kyung, Endo, Shogo, Schuchman, Edward H., Carter, Janet E., Bae, Jae‐sung

“…Alzheimer's disease (AD) is characterized by the deposition of amyloid‐β peptide (Aβ) and the formation of neurofibrillary tangles. Transplantation of bone…”
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Neuronal SphK1 acetylates COX2 and contributes to pathogenesis in a model of Alzheimer’s Disease by Lee, Ju Youn, Han, Seung Hoon, Park, Min Hee, Baek, Bosung, Song, Im-Sook, Choi, Min-Koo, Takuwa, Yoh, Ryu, Hoon, Kim, Seung Hyun, He, Xingxuan, Schuchman, Edward H., Bae, Jae-Sung, Jin, Hee Kyung

“…Although many reports have revealed the importance of defective microglia-mediated amyloid β phagocytosis in Alzheimer’s disease (AD), the underlying mechanism…”
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N-AS-triggered SPMs are direct regulators of microglia in a model of Alzheimer’s disease by Lee, Ju Youn, Han, Seung Hoon, Park, Min Hee, Song, Im-Sook, Choi, Min-Koo, Yu, Eunsoo, Park, Cheol-Min, Kim, Hee-Jin, Kim, Seung Hyun, Schuchman, Edward H., Jin, Hee Kyung, Bae, Jae-sung

“…Sphingosine kinase1 (SphK1) is an acetyl-CoA dependent acetyltransferase which acts on cyclooxygenase2 (COX2) in neurons in a model of Alzheimer’s disease…”
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Immunotherapy targeting plasma ASM is protective in a mouse model of Alzheimer’s disease by Choi, Byung Jo, Park, Min Hee, Park, Kang Ho, Han, Wan Hui, Yoon, Hee Ji, Jung, Hye Yoon, Hong, Ju Yeon, Chowdhury, Md Riad, Kim, Kyung Yeol, Lee, Jihoon, Song, Im-Sook, Pang, Minyeong, Choi, Min-Koo, Gulbins, Erich, Reichel, Martin, Kornhuber, Johannes, Hong, Chang-Won, Kim, Changho, Kim, Seung Hyun, Schuchman, Edward H., Jin, Hee Kyung, Bae, Jae-sung

“…Acid sphingomyelinase (ASM) has been implicated in neurodegenerative disease pathology, including Alzheimer’s disease (AD). However, the specific role of…”
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The cholesterol transporter NPC1 is essential for epigenetic regulation and maturation of oligodendrocyte lineage cells by Kunkel, Thaddeus J., Townsend, Alice, Sullivan, Kyle A., Merlet, Jean, Schuchman, Edward H., Jacobson, Daniel A., Lieberman, Andrew P.

“…The intracellular cholesterol transporter NPC1 functions in late endosomes and lysosomes to efflux unesterified cholesterol, and its deficiency causes…”
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New paradigms for the treatment of lysosomal storage diseases: targeting the endocannabinoid system as a therapeutic strategy by Schuchman, Edward H, Ledesma, Maria D, Simonaro, Calogera M

“…Over the past three decades the lysosomal storage diseases have served as model for rare disease treatment development. While these efforts have led to…”
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Modulation of Dietary Choline Uptake in a Mouse Model of Acid Sphingomyelinase Deficiency by Gaudioso, Ángel, Moreno-Huguet, Pilar, Casas, Josefina, Schuchman, Edward H, Ledesma, María Dolores

“…Acid sphingomyelinase deficiency (ASMD) is a lysosomal storage disorder caused by mutations in the gene-encoding acid sphingomyelinase (ASM). ASMD impacts…”
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Sphingomyelin 16:0 is a therapeutic target for neuronal death in acid sphingomyelinase deficiency by Gaudioso, Ángel, Jiang, Xuntian, Casas, Josefina, Schuchman, Edward H., Ledesma, María Dolores

“…Acid sphingomyelinase deficiency (ASMD) is a lysosomal storage disorder caused by mutations in the SMPD1 gene encoding for the acid sphingomyelinase (ASM)…”
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Recombinant human acid sphingomyelinase as an adjuvant to sorafenib treatment of experimental liver cancer by Savić, Radoslav, He, Xingxuan, Fiel, Isabel, Schuchman, Edward H

“…Hepatocellular carcinoma (HCC) is the most common form of liver cancer and the third leading cause of cancer death worldwide. The only approved systemic…”
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Pentosan Polysulfate: Oral Versus Subcutaneous Injection in Mucopolysaccharidosis Type I Dogs by Simonaro, Calogera M, Tomatsu, Shunji, Sikora, Tracy, Kubaski, Francyne, Frohbergh, Michael, Guevara, Johana M, Wang, Raymond Y, Vera, Moin, Kang, Jennifer L, Smith, Lachlan J, Schuchman, Edward H, Haskins, Mark E

“…We previously demonstrated the therapeutic benefits of pentosan polysulfate (PPS) in a rat model of mucopolysaccharidosis (MPS) type VI. Reduction of…”
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Involvement of the Toll-like receptor 4 pathway and use of TNF-α antagonists for treatment of the mucopolysaccharidoses by Simonaro, Calogera M, Ge, Yi, Eliyahu, Efrat, He, Xingxuan, Jepsen, Karl J, Schuchman, Edward H

“…Enzyme replacement therapy is currently available for three of the mucopolysaccharidoses (MPSs) but has limited effects on the skeletal lesions. We…”
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Pentosan polysulfate: a novel therapy for the mucopolysaccharidoses by Schuchman, Edward H, Ge, Yi, Lai, Alon, Borisov, Yury, Faillace, Meghan, Eliyahu, Efrat, He, Xingxuan, Iatridis, James, Vlassara, Helen, Striker, Gary, Simonaro, Calogera M

“…Pentosan polysulfate (PPS) is an FDA-approved, oral medication with anti-inflammatory and pro-chondrogenic properties. We have previously shown that animal…”
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Apolipoprotein D-mediated preservation of lysosomal function promotes cell survival and delays motor impairment in Niemann-Pick type A disease by Pascua-Maestro, Raquel, Corraliza-Gomez, Miriam, Fadrique-Rojo, Cristian, Ledesma, Maria D., Schuchman, Edward H., Sanchez, Diego, Ganfornina, Maria D.

“…Lysosomal Storage Diseases (LSD) are genetic diseases causing systemic and nervous system dysfunction. The glia-derived lipid binding protein Apolipoprotein D…”
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