Search Results - "Haskins, M. E."

A model of mucopolysaccharidosis IIIB (Sanfilippo syndrome type IIIB): N‐acetyl‐α‐D‐glucosaminidase deficiency in Schipperke dogs by Ellinwood, N. M., Wang, P., Skeen, T., Sharp, N. J. H., Cesta, M., Decker, S., Edwards, N. J., Bublot, I., Thompson, J. N., Bush, W., Hardam, E., Haskins, M. E., Giger, U.

“…Mucopolysaccharidosis III (MPS III) is characterized by lysosomal accumulation of the glycosaminoglycan (GAG) heparan sulphate (HS). In humans, the disease…”
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Clinical characterization of cardiovascular abnormalities associated with feline mucopolysaccharidosis I and VI by Sleeper, M. M., Kusiak, C. M., Shofer, F. S., O’Donnell, P., Bryan, C., Ponder, K. P., Haskins, M. E.

“…Summary Objective: The purpose of this study was to define the cardiovascular abnormalities present in young and adult cats affected with the lysosomal storage…”
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Abnormal neuronal metabolism and storage in mucopolysaccharidosis type VI (Maroteaux-Lamy) disease by Walkley, S. U., Thrall, M. A., Haskins, M. E., Mitchell, T. W., Wenger, D. A., Brown, D. E., Dial, S., Seim, H.

“…Mucopolysaccharidosis (MPS) type VI, also known as Maroteaux–Lamy disease, is an inherited disorder of glycosaminoglycan  catabolism  caused  by  deficient …”
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Gene therapy ameliorates cardiovascular disease in dogs with mucopolysaccharidosis VII by Sleeper, M M, Fornasari, B, Ellinwood, N M, Weil, M A, Melniczek, J, O'Malley, T M, Sammarco, C D, Xu, L, Ponder, K P, Haskins, M E

“…Mucopolysaccharidosis VII (MPS VII) is a lysosomal storage disease caused by deficient beta-glucuronidase (GUSB) activity resulting in defective catabolism of…”
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Mucopolysaccharidosis VII in a Cat Caused by 2 Adjacent Missense Mutations in the GUSB Gene by Wang, P., Sorenson, J., Strickland, S., Mingus, C., Haskins, M.E., Giger, U.

“…Background Mucopolysaccharidoses (MPS) are common lysosomal storage disorders causing typically progressive skeletal and ocular abnormalities. Objectives To…”
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Adeno-associated virus serotypes 9 and rh10 mediate strong neuronal transduction of the dog brain by Swain, G P, Prociuk, M, Bagel, J H, O'Donnell, P, Berger, K, Drobatz, K, Gurda, B L, Haskins, M E, Sands, M S, Vite, C H

“…Canine models have many advantages for evaluating therapy of human central nervous system (CNS) diseases. In contrast to nonhuman primate models, naturally…”
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Enzyme Replacement Therapy in Feline Mucopolysaccharidosis I by Kakkis, E.D., Schuchman, E., He, X., Wan, Q., Kania, S., Wiemelt, S., Hasson, C.W., O'Malley, T., Weil, M.A., Aguirre, G.A., Brown, D.E., Haskins, M.E.

“…Enzyme replacement therapy (ERT) has long been considered an approach to treating lysosomal storage disorders caused by deficiency of lysosomal enzymes. ERT is…”
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Inheritance, biochemical abnormalities, and clinical features of feline Mucolipidosis II: the first animal model of human I-cell disease by Mazrier, H, Van Hoeven, M, Wang, P, Knox, V.W, Aguirre, G.D, Holt, E, Wiemelt, S.P, Sleeper, M.M, Hubler, M, Haskins, M.E

“…Mucolipidosis II (ML II), also called I-cell disease, is a unique lysosomal storage disease caused by deficient activity of the enzyme…”
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Autologous transplantation of retrovirally transduced bone marrow or neonatal blood cells into cats can lead to long-term engraftment in the absence of myeloablation by SIMONARO, C. M, HASKINS, M. E, ABKOWITZ, J. L, BROOKS, D. A, HOPWOOD, J. J, ZHANG, J, SCHUCHMAN, E. H

“…Autologous transplantation of retrovirally transduced bone marrow (BM) or neonatal blood cells was carried out on eight cats (ranging in age from 2 weeks to 12…”
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Molecular Basis of Feline β-Glucuronidase Deficiency: An Animal Model of Mucopolysaccharidosis VII by Fyfe, John C., Kurzhals, Rebeccah L., Lassaline, Mary E., Henthorn, Paula S., Alur, Prasad R.K., Wang, Ping, Wolfe, John H., Giger, Urs, Haskins, Mark E., Patterson, Donald F., Sun, Huaichang, Jain, Sanjeev, Yuhki, Naoya

“…A family of domestic cats was found that exhibited clinical and biochemical abnormalities consistent with mucopolysaccharidosis VII, an autosomal recessive…”
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Cloning of the Canine β-Glucuronidase cDNA, Mutation Identification in Canine MPS VII, and Retroviral Vector-Mediated Correction of MPS VII Cells by Ray, Jharna, Bouvet, Alain, DeSanto, Christopher, Fyfe, John C., Xu, Danbin, Wolfe, John H., Aguirre, Gustavo D., Patterson, Donald F., Haskins, Mark E., Henthorn, Paula S.

“…Mucopolysaccharidosis type VII (MPS VII) is an inherited disease resulting from deficient activity of the lysosomal acid hydrolase β-glucuronidase (GUSB) and…”
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MRI and electrophysiological abnormalities in a case of canine globoid cell leucodystrophy by Cozzi, F., Vite, C. H., Wenger, D. A., Victoria, T., Haskins, M. E.

“…A six‐month‐old West Highland white terrier with progressive, multifocal neurological disease was diagnosed with canine globoid cell leucodystrophy (GCL)…”
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Biochemical, pathological, and clinical response to transplantation of normal bone marrow cells into acid sphingomyelinase-deficient mice by MIRANDA, S. R. P, ERLICH, S, FRIEDRICH, V. L, HASKINS, M. E, GATT, S, SCHUCHMAN, E. H

“…Acid sphingomyelinase knock-out (ASMKO) mice are a model of types A and B Niemann-Pick disease. In the present study, we evaluated whether bone marrow…”
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Nurse leadership: Sustaining a culture of safety by Haskins, Helena E.M, Roets, Lizeth

“…Background: Nurse leaders are essential to manage nursing practices that affect patient safety; therefore, they must create and sustain a sound safety culture…”
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Inherited platelet delta-storage pool disease in dogs causing severe bleeding: an animal model for a specific ADP deficiency by Callan, M B, Bennett, J S, Phillips, D K, Haskins, M E, Hayden, J E, Anderson, J G, Giger, U

“…The nature of a disorder producing moderate to severe bleeding after minor trauma, venipuncture, and surgery was studied in 3 families of American cocker…”
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Biochemical basis of the β-glucuronidase gene defect causing canine mucopolysaccharidosis VII by Ray, J, Scarpino, V, Laing, C, Haskins, ME

“…Mucopolysaccharidosis type VII (MPS VII), or Sly syndrome, is an autosomal recessive lysosomal storage disorder resulting from the deficiency in the activity…”
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Retrovirus vector-mediated correction and cross-correction of lysosomal alpha-mannosidase deficiency in human and feline fibroblasts by Sun, H, Yang, M, Haskins, M E, Patterson, D F, Wolfe, J H

“…Lysosomal alpha-mannosidase (EC 3.2.1.24) is an exoglycosidase in the glycoprotein degradation pathway. A deficiency of this enzyme causes the lysosomal…”
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Articular Chondrocytes from Animals with a Dermatan Sulfate Storage Disease Undergo a High Rate of Apoptosis and Release Nitric Oxide and Inflammatory Cytokines: A Possible Mechanism Underlying Degenerative Joint Disease in the Mucopolysaccharidoses by Simonaro, Calogera M, Haskins, Mark E, Schuchman, Edward H

“…Mucopolysaccharidosis (MPS) Type VI (Maroteaux-Lamy Disease) is the lysosomal storage disease characterized by deficient arylsulfatase B activity and the…”
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Restoration of Normal Lysosomal Function in Mucopolysaccharidosis Type VII Cells by Retroviral Vector-Mediated Gene Transfer by Wolfe, John H., Schuchman, Edward H., Stramm, Lawrence E., Concaugh, Elizabeth A., Haskins, Mark E., Aguirre, Gustavo D., Patterson, Donald F., Desnick, Robert J., Gilboa, Eli

“…Retroviral vectors were constructed containing a rat β-glucuronidase cDNA driven by heterologous promoters. Vector-mediated gene transfer into human and canine…”
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Neonatal Gene Transfer Leads to Widespread Correction of Pathology in a Murine Model of Lysosomal Storage Disease by Daly, Thomas M., Vogler, Carole, Levy, Beth, Haskins, Mark E., Sands, Mark S.

“…For many inborn errors of metabolism, early treatment is critical to prevent long-term developmental sequelae. We have used a gene-therapy approach to…”
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