Does deficiency of arylsulfatase B have a role in cystic fibrosis?
Cystic fibrosis (CF) is associated with mutation and abnormal function of the cystic fibrosis transmembrane conductance regulator (CFTR) that affects cellular chloride transport. Clinically, CF of the lung is associated with excessive accumulation of secretions, including the sulfated glycosaminoglycans, chondroitin sulfate and dermatan sulfate (DS), both of which contain sulfated N-acetylgalactosamine residues. The sulfatase enzymes, which are a highly conserved group of enzymes with high specificity for designated sulfate groups, include arylsulfatase B, a lysosomal enzyme. Arylsulfatase B, also known as N-acetyl galactosamine 4-sulfatase, can degrade DS and chondroitin-4 sulfate. Previously reported data demonstrated diminished activity of arylsulfatase B in lymphoid cell lines of patients with CF compared to normal control subjects. Frequent infections with Pseudomonas, a sulfatase-producing organism, occur in patients with CF, whereas infections with Mycobacterium tuberculosis, which lacks sulfatase activity, are infrequent. Additional investigation to determine if diminished function of arylsulfatase B is a consistent finding in cells of patients with CF may be informative, and may help to correlate the molecular, biochemical, and clinical characteristics of CF.
Tobacman
Joanne K
JK
Department of Internal Medicine, University of Iowa Health Care, Iowa City, IA 52242, USA. joanne-tobacman@uiowa.edu
D001424:Bacterial Infections / Q000378:metabolism; D002809:Chondroitin Sulfates / Q000378:metabolism; D003550:Cystic Fibrosis / Q000201:enzymology*; D003871:Dermatan Sulfate / Q000378:metabolism; D006025:Glycosaminoglycans / Q000378:metabolism; D006801:Humans; D007474:Ion Exchange; D008168:Lung / Q000378:metabolism; D009087:Mucopolysaccharidosis VI / Q000150:complications*; D020051:N-Acetylgalactosamine-4-Sulfatase / Q000378:metabolism; D011552:Pseudomonas Infections / Q000378:metabolism; D012964:Sodium / Q000378:metabolism; D013431:Sulfates / Q000378:metabolism; D014376:Tuberculosis / Q000378:metabolism
D006025:Glycosaminoglycans; D013431:Sulfates; D003871:Dermatan Sulfate; D002809:Chondroitin Sulfates; D012964:Sodium; D020051:N-Acetylgalactosamine-4-Sulfatase