alpha-Galactosidase (Beano)

An enzyme that catalyzes the hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-galactosides including galactose oligosaccharides, galactomannans, and galactolipids.

Also Known As:

Networked: 760 relevant articles (14 outcomes, 40 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Desnick, Robert J: 28 articles (01/2010 - 04/2002)
2.	Schiffmann, Raphael: 23 articles (09/2010 - 02/2002)
3.	Beck, M: 18 articles (09/2010 - 09/2001)
4.	Germain, Dominique P: 17 articles (02/2015 - 01/2002)
5.	Brady, Roscoe O: 17 articles (09/2010 - 02/2002)
6.	Beck, Michael: 16 articles (02/2010 - 11/2002)
7.	Schiffmann, R: 14 articles (04/2009 - 01/2000)
8.	Brady, R O: 14 articles (01/2009 - 01/2000)
9.	Wanner, Christoph: 12 articles (03/2015 - 05/2003)
10.	Sakuraba, Hitoshi: 12 articles (09/2010 - 10/2002)

Related Diseases

1.	Fabry Disease (Fabry's Disease) 06/01/2007 - "Gene therapy for Fabry disease, a deficiency in alpha-galactosidase A (alpha-gal A) activity, has the potential to provide a cure for the disorder with a single treatment. " 07/05/2001 - "Safety and efficacy of recombinant human alpha-galactosidase A--replacement therapy in Fabry's disease." 05/20/2015 - "Due to the importance and the difficulty still present in determining the biochemical diagnosis of Fabry disease (FD), the aim of this study was to establish and compare the biochemical and kinetic properties of alpha-galactosidase A (GLA) in dried blood spots (DBS), plasma and leukocyte samples of FD patients and healthy subjects to evaluate the possible use of these parameters as an auxiliary tool in the diagnosis of this disease. " 02/01/2015 - "The alpha-galactosidase A p.Arg118Cys variant does not cause a Fabry disease phenotype: data from individual patients and family studies." 04/01/2013 - "The study was to explore potential mutations of alpha-galactosidase A (GLA) gene and their correlation with clinic manifestations in three Chinese pedigrees with Fabry disease. "
2.	Flatulence 11/01/2010 - "alpha-Galactosidase from Bacillus megaterium VHM1 and its application in removal of flatulence-causing factors from soymilk." 09/01/2006 - "Reduction of flatus-inducing factors in soymilk by immobilized alpha-galactosidase." 11/01/1994 - "The number of flatulence events per hour was significantly less in the group treated with alpha-galactosidase than placebo over the 6-hour follow-up period (F = 2.87, P = .016). " 09/01/1992 - "A significant reduction of flatulence oligosaccharides took place, which was also explained by an increase of alpha-galactosidase concentration. " 11/01/2002 - "Because of the lack of alpha-galactosidase (alpha-Gal) in the digestive tract, humans are unable to digest these sugars, which consequently induce flatulence. "
3.	Proteinuria 05/01/2005 - "A 52-year-old man, the father of the case one patient, was admitted for renal biopsy because of proteinuria and low levels of WBC alpha-galactosidase. " 02/01/2006 - "Alpha-galactosidase activity should be examined in patients with proteinuria: what have we learned from a family affected with Fabry disease?" 01/01/1999 - "A 45-year-old man who developed proteinuria was diagnosed as having Fabry's disease on the basis of renal histological findings and prominent decreases in alpha-galactosidase A activity in blood leukocytes." 07/01/2010 - "In Fabry nephropathy, alpha-galactosidase deficiency leads to accumulation of glycosphingolipids in all kidney cell types, proteinuria and progressive loss of kidney function. " 03/01/1987 - "A deficiency of alpha galactosidase activity in the leucocytes confirmed the diagnosis of Fabry's disease, although this patient had neither angiokeratoma or proteinuria. "
4.	Cardiomyopathies (Cardiomyopathy) 06/13/2006 - "Mutations in sarcomere protein, PRKAG2, LAMP2, alpha-galactosidase A (GLA), and several mitochondrial genes can cause rare familial cardiomyopathies, but their contribution to increased left ventricular wall thickness (LVWT) in the community is unknown. " 04/01/1986 - "Ordinary medical examinations revealed cardiomyopathy in some asymptomatic females, and the diagnosis of the Fabry heterozygote was established by demonstration of specific inclusion bodies in the biopsied myocardium and low alpha-galactosidase activity in leukocytes. " 02/03/2009 - "These data suggest that treatment of Fabry cardiomyopathy with recombinant alpha-galactosidase A should best be started before myocardial fibrosis has developed to achieve long-term improvement in myocardial morphology and function and exercise capacity." 10/01/2006 - "A unique adult male patient who developed cardiomyopathy was first suspected to have cardiac Fabry disease based on the pathological findings in heart tissues obtained on biopsy, but the alpha-galactosidase activity in his leukocytes was normal and no mutation was detected in the coding region of the alpha-galactosidase gene. " 08/01/2005 - "Fabry disease is an X-linked lysosomal storage disease caused by deficiency of the enzyme alpha-galactosidase A and results in pain, progressive renal impairment, cardiomyopathy, and cerebrovascular disease. "
5.	Pain (Aches) 01/01/2013 - "Symptoms of pain and health status scores did not deteriorate during alpha-galactosidase therapy. " 04/01/2009 - "Three patients were diagnosed as Fabry's disease with clinical characteristics including pain and acroparesthesias, angiokeratoma and decrease of alpha-galactosidase A activity. " 08/01/2005 - "Fabry disease is an X-linked lysosomal storage disease caused by deficiency of the enzyme alpha-galactosidase A and results in pain, progressive renal impairment, cardiomyopathy, and cerebrovascular disease. " 01/01/2007 - "Fabry-Anderson disease is an x-linked deficiency of lysosomal alpha-galactosidase A (GALA), resulting in chronic renal failure, cardiac arrhythmia, hypertrophy, valvular disease, pain (acro-paraesthesiae) and stroke, together with premature mortality. " 01/01/2006 - "Indeed, independent of conservative treatment (pain, proteinuria, chronic renal failure, arterial hypertension, heart failure, etc), enzyme therapy using recombinant alpha-galactosidase (agalsidase) has provided a safe pathophysiological approach, leading to significant organ functional improvement (mainly kidney and heart) and improved quality of life, which parallels tissue GL-3 clearance. "

Related Drugs and Biologics

1.	agalsidase beta
2.	globotriaosylceramide (globotriosylceramide)
3.	Oligosaccharides
4.	Hydrogen
5.	carbohydrase
6.	alpha-Galactosidase (Beano)
7.	Glycosphingolipids
8.	Complementary DNA (cDNA)
9.	agalsidase alfa
10.	Sphingolipids

Related Therapies and Procedures

1.	Enzyme Replacement Therapy
2.	Intravenous Infusions
3.	Enzyme Therapy
4.	Transplantation (Transplant Recipients)
5.	Kidney Transplantation