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Protective protein as an endogenous endothelin degradation enzyme in human tissues.
K Itoh, R Kase, M Shimmoto, A Satake, H Sakuraba, … - J Biol Chem, 1995 - jbc.org ... All these enzyme activities are deficient in galactosialidosis cells(11, 12) . ...Expression of Protective Protein cDNA in Galactosialidosis Cells. ......
Incorporation and degradation of GM1 ganglioside and asialoGM1 ganglioside in cultured fibroblasts …T Kobayashi, N Shinnoh, Y Kuroiwa - Biochim Biophys Acta, 1986 - ncbi.nlm.nih.gov ... hydrolyzed on any day of culture, while fibroblasts from patients with adult GM1gangliosidosis, Morquio disease type B and galactosialidosis hydrolyzed the ......
Human lysosomal protective protein has cathepsin A-like activity distinct from its protective …NJ Galjart, H Morreau, R Willemsen, N Gillemans, … - J Biol Chem, 1991 - ncbi.nlm.nih.gov ... Netherlands. The protective protein was first discovered because of its deficie the metabolic storage disorder galactosialidosis. ......
The relation between human lysosomal beta-galactosidase and its protective proteinAT Hoogeveen, FW Verheijen, H Galjaard - J. Cell Sci, 2000 - jbc.org ... Cultured skin fibroblasts from patients with the lysosomal storage diseasegalactosialidosis lack a 54-kDa protein which is a psor of 32-kDa and 20-kDa ......
Expression of cDNA encoding the human
protective protein
associated with lysosomal beta- …NJ Galjart, N Gillemans, A Harris, GTJ van der … - Cell, 1988 - ncbi.nlm.nih.gov ... is a glycoprotein that associates with lysosomal beta-galactosidase and neuraminidaseand is deficient in the autosomal recessive disorder galactosialidosis. ......
Human genes containing polymorphic trinucleotide repeatsGJ Riggins, LK Lokey, JL Chastain, HA Leiner, SL … - Nature Genetics, 1992 - nature.com ... The presence of a reduced amount of 32-kd "protective" protein is a distinctbiochemical finding in late infantile galactosialidosis. Hum. Genet. ......
Combined deficiency of beta-galactosidase and neuraminidase: Natural history of the disease in the …P Strisciuglio, WS Sly, WE Dodson, WH McAlister, … - Am J Med Genet, 1990 - ncbi.nlm.nih.gov ... We describe the clinical findings over the first 18 years of a patient with a novelphenotype for galactosialidosis, the storage disease produced by the ......
Lysosomal high molecular weight multienzyme complexH Ostrowska, K Krukowska, J Kalinowska, M Orlowska … - Cell Mol. Biol. Lett, 2003 - cmbl.org.pl ... In man, a deficiency of cathepsin A leads to a combined deficiency of
-GAL and NEUR activities, called
galactosialidosis
?. ......
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The information provided in this page is not intended for diagnostic purposes. It is provided for information purposes only. For any further understanding that could lead to the prevention, treatment, and/or cure of the disorders, we recommend that affected individuals seek the advice or counsel of his or her own personal physician(s).
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