Výsledky vyhledávání - "Margaret Z. Jones"

Autor: Cavanagh K, R. A. Fisher, E. J. S. Rathke, J. A. Kelley, Dunstan Rw, Margaret Z. Jones

Publikováno v: Animal Genetics. 17:183-190

Plasma levels of beta-mannosidase activity were measured in the phenotypically normal members of a family of goats in which 12 cases of beta-mannosidosis have been reported. Normal or carrier genotypes were assigned on the basis of these results, and

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8dbe9d7878ea88bff30a4807c6543520
https://doi.org/10.1111/j.1365-2052.1986.tb00737.x

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In utero Hematopoietic Stem Cell Transplantation: A Caprine Model for Prenatal Therapy in Inherited Metabolic Diseases

Autor: Stacey A. Kraemer, Kathryn L. Lovell, Jodi Nichols-Torrez, Margaret Z. Jones, Kawanaa D. Carter, N. Kent Ames, Jeffrey R. Leipprandt, D.J. Sprecher, Darush K. Rahmani

Publikováno v: Fetal Diagnosis and Therapy. 16:13-17

Objectives: We explored the feasibility and efficacy of in utero hematopoietic stem cell transplantation in the caprine animal model system with the objectives of determining procedures for transplantation and establishing methods for detecting engra

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::54d10b95180f0a98075e1e0b4c203d86
https://doi.org/10.1159/000053873

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Differential Activation of p38 Mitogen-activated Protein Kinase Isoforms Depending on Signal Strength

Autor: Gema Alonso, Margaret Z. Jones, Concetta Ambrosino, Angel R. Nebreda

Publikováno v: Journal of Biological Chemistry. 275:40641-40648

We have investigated the ability of the mitogen-activated protein kinase (MAPK) kinase MKK6 to activate different members of the p38 subfamily of MAPKs and found that some MKK6 mutants can efficiently activate p38alpha but not p38gamma. In contrast,

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https://doi.org/10.1074/jbc.m007835200

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Caprine Mucopolysaccharidosis IIID: A Preliminary Trial of Enzyme Replacement Therapy

Autor: John J. Hopwood, Stacey A. Kraemer, John C. Baker, Kevin T. Cavanagh, Barbara King, Erinn Downs-Kelly, Joseph Alroy, Margaret Z. Jones, Rebecca E. Lucas

Publikováno v: Journal of Molecular Neuroscience. 15:251-262

Mucopolysaccharidosis type IIID (MPS IIID) is a lysosomal storage disorder resulting from lack of activity of the lysosomal hydrolase N-acetylglucosamine 6-sulfatase (6S) (EC 3.1.6.14). The syndrome is associated with systemic and central nervous sys

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::942786457c6559e72897e351a5ad76e4
https://doi.org/10.1385/jmn:15:3:251

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Identification of a bovine β-mannosidosis mutation and detection of two β-mannosidase pseudogenes

Autor: Karen H. Friderici, Margaret Z. Jones, Juliann E. Horvath, Jeffrey R. Leipprandt, Hong Chen, Xiao Tan Qiao

Publikováno v: Mammalian Genome. 10:1137-1141

Beta-mannosidase deficiency results in beta-mannosidosis, a severe neurodegenerative lysosomal storage disease identified in cattle, goats, and humans. To more fully understand the molecular pathology of this disease, the mutation associated with bov

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::79e157d068beb3c00a666a6b4a739011
https://doi.org/10.1007/s003359901179

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Amylopectinosis in Fetal and Neonatal Quarter Horses

Autor: J. C. Fyfe, Margaret Z. Jones, F. A. Kennedy, J. A. Render, R. S. Common

Publikováno v: Veterinary Pathology. 36:157-160

Three Quarter Horses, a stillborn filly (horse No. 1), a female fetus aborted at approximately 6 months of gestation (horse No. 2), and a 1-month-old colt that had been weak at birth (horse No. 3), had myopathy characterized histologically by large s

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https://doi.org/10.1354/vp.36-2-157

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Caprine Mucopolysaccharidosis-IIID

Autor: Kent Johnson, Philip J. Boyer, Shyh Shyurng Liour, John J. Hopwood, Robert A. Leedle, Beverly A. L. Levene, Douglas A. Gage, P. Sharp, Margaret Z. Jones, Ralph S. Common, J. A. Render, Rebecca E. Lucas, Kevin T. Cavanagh, Joseph Alroy, Joseph Vorro, Heidi M. Hoard, Charles T. Lowrie

Publikováno v: Journal of Neuropathology and Experimental Neurology. 57:148-157

Several animal models have been developed for the mucopolysaccharidoses (MPSs), a group of lysosomal storage disorders caused by lysosomal hydrolase deficiencies that disrupt the catabolism of glycosaminoglycans (GAG). Among the MPS, the MPS-III (San

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https://doi.org/10.1097/00005072-199802000-00006

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Recombinant Caprine 3H-[N-Acetylglucosamine-6-Sulfatase] and Human 3H-[N-Acetylgalactosamine-4-Sulfatase]: Plasma Clearance, Tissue Distribution, and Cellular Uptake in the Rat

Autor: Leanne K. Brumfield, Margaret Z. Jones, John J. Hopwood, Sharon Byers, Barbara King

Publikováno v: Journal of Molecular Neuroscience. 11:223-232

The use of recombinant lysosomal enzymes for enzyme replacement therapy (ERT) is likely to be a necessary component of effective treatment regimens for lysosomal storage diseases (LSDs). The mechanism and rate of uptake into target cells, rate of dis

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https://doi.org/10.1385/jmn:11:3:223

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Human Mucopolysaccharidosis IIID

Autor: John W. Taylor, Ellsworth C. Alvord, W. Evans, Kevin T. Cavanagh, David A. Thorley-Lawson, Joseph Alroy, Christopher J. Ianelli, Jennifer R. Toone, Derek A. Applegarth, David Sillence, P. Sharp, Ehud Skutelsky, Margaret Z. Jones, Joe C. Rutledge, Albert Arias, Cynthia Mitchell-Herpolsheimer, John J. Hopwood

Publikováno v: Journal of Neuropathology and Experimental Neurology. 56:1158-1167

Mucopolysaccharidosis IIID (MPS IIID) is one of the rarest of the MPS-III syndromes. To date, the clinical manifestations of 10 patients have been reported, the deficient N-acetylglucosamine 6-sulfatase (G6S) enzyme has been purified, and the G6S gen

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https://doi.org/10.1097/00005072-199710000-00010

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BIOCHEMICAL AND MORPHOLOGICAL EXPRESSION OF EARLY PRENATAL CAPRINE β-MANNOSIDOSIS

Autor: Kathryn L. Lovell, Jon S. Patterson, N. K. Ames, F. Matsuura, G. Baeverfjord, Margaret Z. Jones

Publikováno v: Prenatal Diagnosis. 17:551-557

Lysosomal storage diseases associated with early-onset pathological changes may require prenatal therapy to avert the profound effects of the metabolic error on organs, especially the central nervous system. The present investigation determined the e

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https://doi.org/10.1002/(sici)1097-0223(199706)17:6<551::aid-pd109>3.0.co

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