Showing 1-30 of 53 results for "G7163"
Elen A Romão et al.
Clinical nephrology, 79(4), 330-334 (2013-03-30)
Fabry disease is an X-linked inborn error of metabolism, which is caused by the deficiency of α-galactosidase A, leading to progressive accumulation of neutral glycosphingolipids and a-galactosyl breakdown products in most body fluids and several tissues, resulting in the clinical...
Takura Wakinaka et al.
Glycobiology, 23(2), 232-240 (2012-10-24)
Bifidobacterium bifidum is one of the most frequently found bifidobacteria in the intestines of newborn infants. We previously reported that B. bifidum possesses unique metabolic pathways for O-linked glycans on gastrointestinal mucin (Yoshida E, Sakurama H, Kiyohara M, Nakajima M...
Alpha-galactosidase from Mortierella vinacea. Crystallization and properties.
H Suzuki et al.
The Journal of biological chemistry, 245(4), 781-786 (1970-02-25)
Antonio Pisani et al.
Molecular genetics and metabolism, 107(3), 267-275 (2012-09-12)
Anderson-Fabry disease is an X-linked lysosomal storage disorder resulting from the deficiency of the hydrolytic enzyme alpha galactosidase A, with consequent accumulation of globotrioasoyl ceramide in cells and tissues of the body, resulting in a multi-system pathology including end organ...
Aritz Pérez Ruiz de Garibay et al.
Drug design, development and therapy, 6, 303-310 (2012-11-03)
Gene-mediated enzyme replacement is a reasonable and highly promising approach for the treatment of Fabry disease (FD). The objective of the present study was to demonstrate the potential applications of solid lipid nanoparticle (SLN)-based nonviral vectors for the treatment of...
Neelesh Singh et al.
Food chemistry, 142, 430-438 (2013-09-05)
Cicer α-galactosidase was immobilized onto functionalized graphene with immobilization efficiency of 84% using response surface methodology (Box-Behnken design). The immobilized enzyme had higher thermal stability than the soluble one, attractive for industrial applications. Immobilization of the enzyme lowered the Km...
Camilla Tøndel et al.
Journal of the American Society of Nephrology : JASN, 24(1), 137-148 (2013-01-01)
The effect of early-onset enzyme replacement therapy on renal morphologic features in Fabry disease is largely unknown. Here, we evaluated the effect of 5 years of treatment with agalsidase alfa or agalsidase beta in 12 consecutive patients age 7-33 years...
Olaf A Bodamer et al.
Current protocols in human genetics, Chapter 17, Unit17-Unit17 (2013-04-19)
Fabry disease (FD) is an X-linked lysosomal storage disorder due to deficiency of alpha galactosidase A (GLA). Progressive, intralysosomal accumulation of neutral glycosphingolipids in endothelial cells and podocytes leads to multi-organ involvement in affected males and to a lesser extent...
Huma Mamun Mahmud
JPMA. The Journal of the Pakistan Medical Association, 64(2), 189-194 (2014-03-20)
Fabry's is a progressive, destructive and life threatening disease which reduces significantly life expectancy of the affected individual. It is a genetic disorder of X-linked inheritance caused by deficiency of lysosomal enzyme alpha-galactosidase A resulting in progressive accumulation of glycosphingolipids...
Red meat allergy in Sweden: association with tick sensitization and B-negative blood groups.
Carl Hamsten et al.
The Journal of allergy and clinical immunology, 132(6), 1431-1434 (2013-10-08)
Hongwei Gao et al.
Artificial cells, nanomedicine, and biotechnology, 41(1), 32-36 (2012-10-04)
Enzymatical conversion of A or B RBCs into group O RBCs (ECORBCs) was achieved by using α-N-acetylgalactosaminidase and α-galactosidase, respectively. Now, we initiated AB to O-RBC conversion by using these two enzymes together. But α-N-acetylgalactosaminidase and α-galactosidase's preserving and their...
K Schmid et al.
European journal of biochemistry, 67(1), 95-104 (1976-08-01)
The utilization by Escherichia coli K12 of raffinose as sole carbon source depends on a new raffinose transport system, an invertase and an alpha-galactosidase specified by the Raf-plasmid D1021. The alpha-galactosidase was purified to homogeneity from a mutant strain with...
Romain Merceron et al.
The Journal of biological chemistry, 287(47), 39642-39652 (2012-09-27)
The α-galactosidase AgaA from the thermophilic microorganism Geobacillus stearothermophilus has great industrial potential because it is fully active at 338 K against raffinose and can increase the yield of manufactured sucrose. AgaB has lower affinity for its natural substrates but...
Successful desensitization to agalsidase beta after anaphylaxis.
Neetu Talreja et al.
Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology, 112(1), 71-72 (2013-12-18)
Clinical problem-solving. In the thick of it.
Deepak A Rao et al.
The New England journal of medicine, 368(18), 1732-1738 (2013-05-03)
B E Smid et al.
Molecular genetics and metabolism, 108(2), 132-137 (2013-01-22)
Enzyme replacement therapy for Fabry disease, consisting of biweekly infusions, interferes daily life. Home treatment proved beneficial. We evaluated a previously reported home treatment algorithm aiming to shorten the period of in-hospital infusions, while ascertaining patient safety. Retrospective analysis on...
Costanza Simoncini et al.
The neurologist, 18(6), 413-414 (2012-11-02)
Fabry disease (FD) is a rare, X-linked lysosomal storage disorder with multiorgan involvement. FD is caused by a partial or total deficit of α-galactosidase A enzyme, which is responsible for the accumulation of glycosphingolipids in a variety of cell types....
Dominique P Germain et al.
Orphanet journal of rare diseases, 7, 91-91 (2012-11-28)
Fabry disease (FD) is a genetic disorder resulting from deficiency of the lysosomal enzyme α-galactosidase A (α-Gal A), which leads to globotriaosylceramide (GL-3) accumulation in multiple tissues. We report on the safety and pharmacodynamics of migalastat hydrochloride, an investigational pharmacological...
Masahisa Kobayashi et al.
Molecular genetics and metabolism, 107(4), 711-715 (2012-11-14)
Fabry disease is an X-linked lysosomal disorder resulting from mutations in the α-galactosidase A (GLA) gene. Recent reports described that the E66Q mutation of GLA is not a disease-causing mutation. However, no pathological study was reported. We carried out pathological...
Shinkyu Choi et al.
Arteriosclerosis, thrombosis, and vascular biology, 34(1), 81-89 (2013-10-26)
Globotriaosylceramide (Gb3) induces KCa3.1 downregulation in Fabry disease (FD). We investigated whether Gb3 induces KCa3.1 endocytosis and degradation. KCa3.1, especially plasma membrane-localized KCa3.1, was downregulated in both Gb3-treated mouse aortic endothelial cells (MAECs) and human umbilical vein endothelial cells. Gb3-induced...
Yulong Tang et al.
Journal of microbiology (Seoul, Korea), 50(6), 994-1002 (2013-01-01)
Catabolite control protein A (CcpA) is the major transcriptional regulator in carbon catabolite repression in several Gram-positive bacteria. We attempted to characterize the role of a CcpA homologue of Streptococcus suis type 2 in sugar metabolism and virulence. Addition of...
Rui Quinta et al.
Gene, 536(1), 97-104 (2013-12-18)
Fabry disease is an X-linked lysosomal storage disease (LSD) caused by deficient activity of α-Galactosidase A (α-Gal A). As a result, glycosphingolipids, mainly globotriaosylceramide (Gb3), progressively accumulate in body fluids and tissues. Studies aiming at the identification of secondary lipid...
Xiao-Liang Pan et al.
The journal of physical chemistry. B, 117(2), 484-489 (2012-12-20)
The enzyme α-galactosidase (α-GAL), a member of glycoside hydrolase family 27, catalyzes the removal of a nonreducing terminal α-galactose residue from polysaccharides, glycolipids, and glycopeptides. α-GAL is believed to have the double displacement retaining reaction mechanism. In this work, the...
K Wyatt et al.
Health technology assessment (Winchester, England), 16(39), 1-543 (2012-10-24)
To determine natural history and estimate effectiveness and cost of enzyme replacement therapy (ERT) and substrate replacement therapy (SRT) for patients with Gaucher disease, Fabry disease, mucopolysaccharidosis type I (MPS I), mucopolysaccharidosis type II (MPS II), Pompe disease and Niemann-Pick...
Saskia M Rombach et al.
PloS one, 7(10), e47805-e47805 (2012-10-25)
Enzyme replacement therapy (ERT) with alpha-Galactosidase A (aGal A) may cause antibody (AB) formation against aGal A in males with Fabry disease (FD). Anti agalsidase ABs negatively influence globotriaosylceramide (Gb3) reduction. We investigated the impact of agalsidase AB on Gb3...
Colm O'Mahony et al.
International journal of STD & AIDS, 25(5), 378-379 (2013-10-10)
Many patients have a few scattered angiokeratoma and we reassure them that this it is normal; however, if they are numerous, Fabry disease should be considered and the family history should be checked.
M Benelmekki et al.
Colloids and surfaces. B, Biointerfaces, 101, 370-375 (2012-09-27)
Biomagnetic immobilization of histidine-rich proteins based on the single-step affinity adsorption of transition metal ions continues to be a suitable practice as a cost effective and a up scaled alternative to the to multiple-step chromatographic separations. In our previous work...
Giovanni Di Nardo et al.
BMC gastroenterology, 13, 142-142 (2013-09-26)
Gas-related symptoms represent very common complaints in children. The reduction of gas production can be considered as a valuable target in controlling symptoms. α-galactosidase has been shown to reduce gas production and related symptoms in adults. To evaluate the efficacy...
Sema Kalkan Uçar et al.
Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy, 16(6), 560-565 (2012-11-30)
Fabry 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...
[Interrelation of alpha-D-fucosidase and alpha-D-galactosidase activities in man and animals].
G Ia Vidershaîn et al.
Doklady Akademii nauk SSSR, 231(2), 486-488 (1976-01-01)

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