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:

Beano; alpha-Galactosidase A; Melibiase; alpha-D-Galactopyranosidase; alpha-D-Galactosidase; alpha-Galactisidase; alpha-Galactosidases; alpha D Galactopyranosidase; alpha D Galactosidase; alpha Galactisidase; alpha Galactosidase; alpha Galactosidase A; alpha Galactosidases; alpha-D-galactoside galactohydrolase

Networked: 1003 relevant articles (14 outcomes, 45 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Enzymes and Coenzymes: 1
- Enzymes: 152586
  - Hydrolases: 2166
    - Glycoside Hydrolases: 871
      - Galactosidases: 1473
        alpha-Galactosidase: 1003
        agalsidase alfa: 147
        agalsidase beta: 114
        alpha-galactosidase I: 1
        4-methylumbelliferyl alpha-galactosidase
        alpha-galactosidase II
        Carnobacterium piscicola AgaA protein
        human GLA protein
        N-acetyl-alpha-D-galactosaminyloligosaccharidase
        trihexosylceramide alpha-galactosidase

Experts

1.	Desnick, Robert J: 31 articles (09/2020 - 04/2002)
2.	Schiffmann, Raphael: 23 articles (09/2010 - 02/2002)
3.	Germain, Dominique P: 19 articles (06/2017 - 01/2002)
4.	Brady, Roscoe O: 19 articles (01/2016 - 02/2002)
5.	Beck, M: 18 articles (09/2010 - 09/2001)
6.	Wanner, Christoph: 17 articles (01/2022 - 05/2003)
7.	Beck, Michael: 16 articles (02/2010 - 11/2002)
8.	Sakuraba, Hitoshi: 15 articles (01/2022 - 10/2002)
9.	Schiffmann, R: 14 articles (04/2009 - 01/2000)
10.	Brady, R O: 14 articles (01/2009 - 01/2000)

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. " 01/01/2021 - "In this pilot, single-arm study (NCT02800070), five adult males with Type 1 (classical) phenotype Fabry disease were infused with autologous lentivirus-transduced, CD34+-selected, hematopoietic stem/progenitor cells engineered to express alpha-galactosidase A (α-gal A). " 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 01/01/2020 - "Due to a lack of alpha-galactosidases in the stomachs of humans and other monogastric animals, these RFOs are not metabolized and are passed to the intestines to be processed by gut bacteria leading to distressing 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. "
3.	Pain (Aches) 01/01/2013 - "Symptoms of pain and health status scores did not deteriorate during alpha-galactosidase therapy. " 01/01/2016 - "alpha-galactosidase A-deficient mice seem to model human Fabry disease and may be helpful when studying the pathophysiology of Fabry-associated pain." 01/01/2016 - "Comprehensive and differential long-term characterization of the alpha-galactosidase A deficient mouse model of Fabry disease focusing on the sensory system and pain development." 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. " 05/12/2023 - "Pain in Fabry disease (FD) is generally accepted to result from neuronal damage in the peripheral nervous system as a consequence of excess lipid storage caused by alpha-galactosidase A (α-Gal A) deficiency. "
4.	Chronic Kidney Failure (Chronic Renal Failure) 01/01/2002 - ": Fabry disease results from an X-linked deficiency of lysosomal alpha-galactosidase A and is a rare cause of end-stage renal disease. " 01/01/2020 - "Previous screening studies have shown that a significant proportion of end-stage renal disease (ESRD) patients receiving hemodialysis (HD) have unsuspected FD, and the prevalence of low alpha-galactosidase A (αGLA) enzyme activity in these patients is higher than that in the normal population. " 03/01/2007 - "Deficiency of lysosomal alpha-galactosidase A (alpha-Gal A) in Fabry disease results in cellular accumulation of globotriaosylceramide (Gl3), often leading to end-stage renal failure. " 05/01/2009 - "In male patients with Fabry disease, an X-linked disorder of glycosphingolipid metabolism caused by deficient activity of the lysosomal enzyme alpha-galactosidase A, kidney dysfunction becomes apparent by the third decade of life and invariably progresses to ESRD without treatment. " 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. "
5.	Inborn Genetic Diseases (Disease, Hereditary) 01/01/2005 - "Defect in the basic alpha-galactosidase molecule implies genetic change, which can be a predisposing factor for the development of atypical and typical forms of this genetic disease. " 01/01/2020 - "Fabry disease is an X-linked lysosomal storage genetic disorder associated with over 1000 mutations in the alpha-galactosidase-A gene region. " 03/01/2011 - "Fabry disease, a genetic disorder caused by deficiency of alpha-galactosidase A activity, is associated with CV dysfunction. " 07/01/2010 - "Fabry disease is an X-linked genetic disorder caused by a deficiency of alpha-galactosidase A (GLA) activity. " 03/01/2010 - "Fabry disease is a genetic disorder caused by a deficiency of alpha-galactosidase, exhibiting a wide clinical spectrum, from the early-onset severe 'classic' form to the late-onset mild 'variant' one. "

Related Drugs and Biologics

1.	agalsidase beta
2.	Enzymes
3.	Hydrogen
4.	globotriaosylceramide (globotriosylceramide)
5.	Oligosaccharides
6.	Peptide Hydrolases (Proteases)
7.	carbohydrase
8.	Glycosphingolipids
9.	alpha-Galactosidase (Beano)
10.	alpha-N-Acetylgalactosaminidase (N-Acetyl-alpha-D-Galactosaminidase)

Related Therapies and Procedures

1.	Enzyme Replacement Therapy
2.	Intravenous Infusions
3.	Therapeutics
4.	Enzyme Therapy
5.	Conservative Treatment