Cerebroside-Sulfatase (Arylsulfatase A)

An enzyme that catalyzes the hydrolysis of cerebroside 3-sulfate (sulfatide) to yield a cerebroside and inorganic sulfate. A marked deficiency of arylsulfatase A, which is considered the heat-labile component of cerebroside sulfatase, has been demonstrated in all forms of metachromatic leukodystrophy (LEUKODYSTROPHY, METACHROMATIC). EC 3.1.6.8.

Also Known As:

Networked: 324 relevant articles (7 outcomes, 15 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Gieselmann, Volkmar: 27 articles (09/2015 - 01/2003)
2.	Gieselmann, V: 14 articles (02/2010 - 03/2000)
3.	Matzner, Ulrich: 11 articles (09/2015 - 01/2005)
4.	Eckhardt, Matthias: 8 articles (09/2015 - 06/2006)
5.	Fogh, Jens: 7 articles (06/2012 - 05/2005)
6.	Lüllmann-Rauch, R: 7 articles (01/2006 - 05/2001)
7.	D'Hooge, Rudi: 6 articles (07/2015 - 09/2007)
8.	Lüllmann-Rauch, Renate: 6 articles (09/2011 - 05/2005)
9.	Matzner, U: 6 articles (01/2006 - 05/2000)
10.	Stroobants, Stijn: 5 articles (07/2015 - 06/2008)

Related Diseases

1.	Metachromatic Leukodystrophy (Sulfatide Lipidosis) 07/01/1972 - "An improved method for the determination of leukocyte arylsulfatase A and its application to the diagnosis of metachromatic leukodystrophy in homozygous and heterozygous states." 05/20/2011 - "Preclinical studies in mouse models of metachromatic leukodystrophy, however, showed that arylsulfatase A (ASA) is able to cross the BBB to some extent, thus reducing lysosomal storage in brain microglial cells. " 12/01/2006 - "In this proof of concept study, we explore whether glial precursors derived from murine ES cells (ESGPs) and engineered to overexpress human arylsulfatase A (hASA) can cross-correct the metabolic defect in an animal model of metachromatic leukodystrophy (MLD). " 11/01/2005 - "In this multicentre study, we examined the prevalence of two mutations in the arylsulfatase A (ARSA) gene, i.e., c.459+1G>A and p.P426L, in 384 unrelated European patients presenting with different types of metachromatic leukodystrophy (MLD). " 12/23/1985 - "Studies in metachromatic leukodystrophy: XV. Purification of normal and mutant arylsulfatase A from human liver."
2.	Neoplasms (Cancer) 12/31/1991 - "More detailed statistical analysis of the results indicates that determination of the serum concentration of arylsulfatase A might be helpful in the diagnosis of lung (59% sensitivity, 82% specificity) and central nervous system cancer (60% sensitivity, 82% specificity). " 06/01/2000 - "To understand the significance of any changes in the glycosylation of arylsulfatase A in cancer, it is important to know the structure of its carbohydrate component in normal tissue. " 06/01/2000 - "It has been shown that the concentration of arylsulfatase A increases in the body fluids of patients with some forms of cancer, and the carbohydrate component of arylsulfatase A synthesized in tumor tissues and transformed cells undergoes increased sialylation, phosphorylation and sulfation. " 03/01/1997 - "To understand the significance of any changes in glycosylation of arylsulfatase A in cancer, it is important to know the structure of its carbohydrate component in normal tissue. " 03/01/1997 - "It has been shown that the concentration of arylsulfatase A increases in the body fluids of patients with some forms of cancer and the carbohydrate component of arylsulfatase A synthesized in tumor tissues and transformed cells undergoes increased sialylation, phosphorylation and sulfation. "
3.	Nervous System Diseases (Neurological Disorders) 09/01/1991 - "Reduced activity of arylsulfatase A and predisposition to neurological disorders: analysis of 140 pediatric patients." 07/01/2014 - "Arylsulfatase A (ASA) deficiency is the cause of metachromatic leucodystrophy (MLD), a lysosomal storage disease associated with severe neurological disorders. " 04/01/2009 - "To assess the efficacy of long-term ERT in metachromatic leukodystrophy (MLD), an LSD with prevailing nervous system disease, we treated immunotolerant arylsulfatase A (ASA) knockout mice with 52 doses of either 4 or 50 mg/kg recombinant human ASA (rhASA). "
4.	Mucopolysaccharidosis VI (Syndrome, Maroteaux-Lamy) 01/01/1981 - "An improved method has been developed for the detection of heterozygotes for feline and human mucopolysaccharidosis VI. Arylsulfatase-A and -B activities were assayed in leukocyte extracts following separation of the enzymes by batch chromatography on DEAE cellulose. " 10/31/1991 - "The enzyme activity of arylsulfatase A and arylsulfatase B was studied in Epstein-Barr virus-transformed lymphoid cell lines established from control individuals and patients affected with metachromatic leukodystrophy, mucopolysaccharidosis type VI (or Maroteaux-Lamy syndrome) and multiple sulfatase deficiency. "
5.	Memory Disorders (Memory Loss) 01/01/2008 - "Transplanted MLD mice showed an improved arylsulfatase A (ARSA) activity and a significant amelioration of sulfatide metabolism, neurodegeneration and motor-learning/memory deficits. "

Related Drugs and Biologics

1.	Sulfoglycosphingolipids (Sulfatides)
2.	Enzymes
3.	Lysergic Acid Diethylamide (LSD)
4.	DEAE-Cellulose
5.	Complementary DNA (cDNA)
6.	N-Acetylgalactosamine-4-Sulfatase (Arylsulfatase B)
7.	Arylsulfatases
8.	beta-Galactosidase (Lactaid)
9.	Molecular Chaperones (Chaperone, Molecular)
10.	Interleukin-8 (Interleukin 8)

Related Therapies and Procedures

1.	Enzyme Replacement Therapy
2.	Transplants (Transplant)
3.	Tissue Therapy (Cell Therapy)
4.	Intravenous Injections
5.	Denervation