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Glucosylceramidase (Glucocerebrosidase)

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A glycosidase that hydrolyzes a glucosylceramide to yield free ceramide plus glucose. Deficiency of this enzyme leads to abnormally high concentrations of glucosylceramide in the brain in GAUCHER DISEASE. EC 3.2.1.45.

Also Known As:

Glucocerebrosidase; Acid beta-Glucosidase; Glucosylceramide beta-Glucosidase; Glucocerebroside beta-Glucosidase; Glucosyl Ceramidase; Glucosylsphingosine Glucosyl Hydrolase; beta-Glucocerebrosidase; Acid beta Glucosidase; Ceramidase, Glucosyl; Glucocerebroside beta Glucosidase; Glucosyl Hydrolase, Glucosylsphingosine; Glucosylceramide beta Glucosidase; Hydrolase, Glucosylsphingosine Glucosyl; beta Glucocerebrosidase; beta-Glucosidase, Acid; beta-Glucosidase, Glucocerebroside; beta-Glucosidase, Glucosylceramide; D-Glucosyl-N-acylsphingosine glucosylhydrolase

Networked: 1049 relevant articles (23 outcomes, 70 trials/studies)

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Bio-Agent Context: Research Results

Enzymes and Coenzymes
- Enzymes: 34502
  - Hydrolases: 639
    - Glycoside Hydrolases: 275
      - Glucosidases: 578
        Glycosylceramidase: 19
        Glucosylceramidase: 1049
        imiglucerase: 123
        alglucerase: 60
        human Velaglucerase alfa

Experts

1.	Sidransky, Ellen: 59 articles (08/2015 - 01/2002)
2.	Grabowski, Gregory A: 26 articles (03/2013 - 01/2003)
3.	Futerman, Anthony H: 24 articles (03/2015 - 11/2002)
4.	Zimran, Ari: 18 articles (07/2015 - 01/2003)
5.	Schapira, Anthony H V: 16 articles (12/2015 - 04/2012)
6.	Elstein, Deborah: 16 articles (07/2015 - 01/2003)
7.	Tayebi, Nahid: 16 articles (06/2014 - 08/2002)
8.	Mehta, Atul: 15 articles (04/2015 - 09/2004)
9.	Aerts, Johannes M F G: 15 articles (04/2015 - 01/2004)
10.	Goker-Alpan, Ozlem: 15 articles (08/2012 - 01/2004)

Related Diseases

1.	Gaucher Disease (Gaucher's Disease) 05/25/1996 - "Regular intravenous administration of modified glucocerebrosidase, derived from human placentae or CHO cells, has proven highly effective in reducing disease manifestations in patients with Gaucher's disease. " 02/01/1995 - "We conclude from these studies that rapid intracellular degradation of administered glucocerebrosidase is the prime factor responsible for the high dose required for effective treatment of Gaucher's disease." 11/01/2000 - "Finally, our findings may help to better understand the molecular basis of Gaucher disease, the human lysosomal disease resulting from deficiency in glucocerebrosidase." 11/01/1993 - "Novel procedures must be developed to deliver glucocerebrosidase to the nervous system so that patients with type 2 and type 3a Gaucher disease can be helped. " 05/01/2015 - "The determination of deficient β-glucocerebrosidase activity in leukocytes or fibroblasts by enzymatic assay is the gold standard for the diagnosis of Gaucher disease. "
2.	Neurologic Manifestations (Neurological Manifestations) 10/01/2007 - "Profound deficiency of acid beta-glucosidase caused by disabling mutations is additionally associated with neurological manifestations in the less common type 2 and type 3 Gaucher diseases. " 07/01/2004 - "Severe deficiency of glucocerebrosidase caused by disabling mutation is additionally associated with neurological manifestations in the less common type 2 and type 3 Gaucher diseases. " 02/01/2004 - "Severe deficiency of glucocerebrosidase caused by severe mutations is additionally associated with neurological manifestations in the less common type 2 and type 3 GD subtypes. " 04/01/1998 - "A novel mutation of the beta-glucocerebrosidase gene associated with neurologic manifestations in three sibs." 03/01/2014 - "The clinical presentation of Gaucher disease (GD), an inherited lysosomal storage disorder caused by the deficient activity of the lysosomal enzyme glucocerebrosidase, is highly variable, and three clinical types are distinguished based upon the presence of neurologic symptoms. "
3.	Lysosomal Storage Diseases (Lysosomal Storage Disease) 08/01/2015 - "Gaucher disease (GD) is the most common lysosomal storage disease resulting from mutations in the lysosomal enzyme glucocerebrosidase (GCase). " 04/01/2015 - "Homozygous mutations in the glucocerebrosidase (GBA) gene result in Gaucher disease (GD), the most common lysosomal storage disease. " 02/01/2015 - "Gaucher disease, the most common lysosomal storage disease, is caused by a recessively inherited deficiency in glucocerebrosidase and subsequent accumulation of toxic lipid substrates. " 01/28/2015 - "Deficiency of the lysosomal glycoside hydrolase glucocerebrosidase (GCase) leads to abnormal accumulation of glucosyl ceramide in lysosomes and the development of the lysosomal storage disease known as Gaucher's disease. " 02/01/2014 - "Gaucher's disease (GD), an inherited metabolic disorder caused by mutations in the glucocerebrosidase gene (GBA), is the most common lysosomal storage disease. "
4.	Thrombocytopenia (Thrombopenia) 07/01/2004 - "Partial deficiency of glucocerebrosidase is associated with parenchymal disease of the liver, spleen, and bone marrow with concomitant anaemia and thrombocytopenia in non-neuronopathic, type 1, Gaucher disease. " 02/01/2004 - "Partial deficiency of acid beta-glucosidase is associated with parenchymal disease of the liver, spleen, and bone marrow with concomitant anemia and thrombocytopenia in non-neuronopathic, type 1 GD. " 11/01/2001 - "An Ashkenazi Jewish woman with Gaucher disease has a huge spleen and severe thrombocytopenia; her older brother, homozygous for the same 1226G glucocerebrosidase mutation, is found on routine examination to have a barely palpable spleen tip. " 10/03/1997 - "A 26-year-old Bedouin with moderate thrombocytopenia and enlarged spleen and liver was diagnosed as having type I Gaucher disease based on the presence of Gaucher cells in the bone marrow biopsy and enzymatic determination of glucocerebrosidase activity. " 07/01/2011 - "Type 1 Gaucher disease (GD) results from inherited β-glucocerebrosidase gene mutations, leading to anemia, thrombocytopenia, splenomegaly, hepatomegaly and skeletal disease. "
5.	Splenomegaly 05/01/2015 - "Glucosylceramidase activity above levels required for clearance of glucosylceramide from tissues resulted in reversal of splenomegaly, reduced Gaucher cell infiltration and a restoration of hematological parameters. " 10/03/1997 - "A 26-year-old Bedouin with moderate thrombocytopenia and enlarged spleen and liver was diagnosed as having type I Gaucher disease based on the presence of Gaucher cells in the bone marrow biopsy and enzymatic determination of glucocerebrosidase activity. " 07/01/2011 - "Type 1 Gaucher disease (GD) results from inherited β-glucocerebrosidase gene mutations, leading to anemia, thrombocytopenia, splenomegaly, hepatomegaly and skeletal disease. "

Related Drugs and Biologics

1.	Mannose (D-Mannose)
2.	imiglucerase (Cerezyme)
3.	alglucerase (Ceredase)
4.	Lysergic Acid Diethylamide (LSD)
5.	Glucosylceramidase (Glucocerebrosidase)
6.	Complementary DNA (cDNA)
7.	Glucose (Dextrose)
8.	Synucleins
9.	DNA (Deoxyribonucleic Acid)
10.	conduritol epoxide

Related Therapies and Procedures

1.	Enzyme Replacement Therapy
2.	Enzyme Therapy
3.	Intravenous Infusions
4.	Complementary Therapies (Alternative Medicine)
5.	Therapeutics