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[Fabry disease (alpha-galactosidase deficiency)].
Nihon rinsho. Japanese journal of clinical medicine, 1996Fabry disease is an X-linked glycosphingolipid storage disorder resulting from a deficiency of lysosomal alpha-galactosidase (alpha-Gal; EC 3.2.1.22). Classical form patients, with clinical manifestations of generalized angiopathy of early onset, usually show no detectable alpha-Gal activity.
T, Okumiya, H, Sakuraba
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Pseudodeficiency of alpha-galactosidase A.
Clinical genetics, 1982Apparent deficiency of alpha-galactosidase A was observed in a 51-year-old, clinically healthy male, with no clinical symptoms of Fabry disease, and without excess urinary excretion of ceramide trihexoside. The deficiency, which was similar to that found in Fabry disease patients, could be demonstrated using both synthetic and natural substrates.
G, Bach +3 more
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[Microbial alpha-galactosidase (a review)].
Prikladnaia biokhimiia i mikrobiologiia, 1982The review discusses properties, distribution and potential use of microbial alpha-galactosidase (alpha-D-galactoside galactohydrolase, EC 3.2.1.22), the enzyme catalyzing degradation of alpha-D-galactoside bonds. Recent years have witnessed many publications describing microbial alpha-galactosidase which, in contrast to the similar enzyme from higher ...
I V, Ulezlo, O M, Zaprometova
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Rinsho byori. The Japanese journal of clinical pathology, 1997
Fabry disease is characterized by a deficiency of lysosomal alpha-galactosidase (alpha-Gal) and the accumulation of glycosphingolipid (e.g. predominantly globotriaosylceramide) in various tissues, mainly in lysosomes of the vascular endothelium. This disorder is currently classified into two clinical phenotypes; classical severe type and atypical ...
T, Okumiya +3 more
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Fabry disease is characterized by a deficiency of lysosomal alpha-galactosidase (alpha-Gal) and the accumulation of glycosphingolipid (e.g. predominantly globotriaosylceramide) in various tissues, mainly in lysosomes of the vascular endothelium. This disorder is currently classified into two clinical phenotypes; classical severe type and atypical ...
T, Okumiya +3 more
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Alpha‐Galactosidase A Activity Levels in Turkish Male Hemodialysis Patients
Therapeutic Apheresis and Dialysis, 2012AbstractFabry disease is an X‐linked lysosomal storage disorder due to deficient activity of alpha‐galactosidase A (α‐Gal A) leading to renal insufficiency in males. The aim of present study was to investigate the level of α‐Gal A activity and to determine the prevalence of Fabry disease in a Turkish male hemodialysis population. The activity of plasma
Sozmen, Eser +4 more
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[Alpha-galactosidase producers among yeast cultures].
Prikladnaia biokhimiia i mikrobiologiia, 1981The capacity for biosynthesis of alpha-galactosidase was examined in 89 yeast cultures belonging to 21 genera during submerged cultivation on a medium containing dry whey. This capacity was found only in one genus, namely Schwanniomyces. Optimal cultivation conditions were selected for the strain Schw.
I V, Ulezlo +2 more
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Multidimensional computational strategies enhance the thermostability of alpha-galactosidase
International Journal of Biological MacromoleculesAlpha-Galactosidase has significant industrial application value in food processing, animal nutrition and medical applications. Microbial-derived α-galactosidases predominate industrial implementation due to high productivity, yet their inherent thermal instability necessitates systematic protein engineering.
Youfeng Zou +4 more
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[Thermal inactivation of alpha-galactosidase from Penicillium canescens].
Ukrains'kyi biokhimichnyi zhurnal (1999 ), 2011The kinetics and mechanism of thermal inactivation of Penicillium canescens alpha-galactosidase in the temperature range of 55-65 degrees C have been studied. The kinetic scheme of alpha-galactosidase thermal inactivation was proposed which included the reversible dissociation of active hexamers into associating monomers and irreversible denaturation ...
N V, Borzova, L D, Varbanets
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Alpha-galactosidase gene cloning from aspergillus fumigatus
2020oz ASPERGILLUS FUMTGATUSTAN ALFA-GALAKTOSIDAZ GENI KLONLANMASI Yalçındağ, Banu Yüksek Lisans, Gıda Mühendisliği Bölümü Tez Danışmanı: Doç. Dr. Zümrüt Begüm Ögel Yardımcı Tez Danışmam: Doç. Dr. Ufuk Bakır Ocak 2002, 88 sayfa Bu çalışmada Aspergillus fumigatus IMI 385708 a-galaktosidaz geni üzerinde moleküler klonlama çalışmaları gerçekleştirilmiştir ...
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