Document Detail


Reduced Arylsulfatase B activity in leukocytes from cystic fibrosis patients.
MedLine Citation:
PMID:  22550062     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The enzyme Arylsulfatase B (ARSB; N-acetylgalactosamine-4-sulfatase) removes 4-sulfate groups from chondroitin-4-sulfate and dermatan sulfate and is required for the degradation of these sulfated glycosaminoglycans (sGAGs). Since these GAGs accumulate in patients with Cystic Fibrosis (CF), we investigated the activity of ARSB in leukocytes of patients with CF, to consider if reduced activity of ARSB might contribute to the pathophysiology of CF. Previous cell-based experiments had demonstrated that when the deficiency of the cystic fibrosis transmembrane regulator (CFTR) was corrected in bronchial epithelial cells, the ARSB activity increased significantly. De-identified, citrated blood samples were collected from 16 children with CF and 31 control subjects, seen in the Pediatric Clinic at Rush University Medical Center. Polymorphonuclear leukocytes (PMN) and mononuclear cell (MC) populations were separated by density gradient, and blinded determinations of ARSB activity were performed using the exogenous substrate 4-methylumbilliferyl sulfate. Interleukin-6 was measured in the plasma samples by ELISA. ARSB activity was significantly less in the PMN and MC from the CF patients than controls (P < 0.0001, unpaired t-test, two-tailed). Interleukin-6 levels in plasma were significantly greater in the CF population (P < 0.001). Mean age, age range, and male:female ratio of CF patients and controls were similar, and no association of ARSB activity with age, gender, or CFTR genotype was evident. Since recombinant human ARSB is used successfully for replacement therapy in Mucopolysaccharidosis VI, it may be useful to restore ARSB activity to normal levels and increase degradation of sulfated GAGs in CF patients.
Authors:
Girish Sharma; Jenifer Burke; Sumit Bhattacharyya; Neha Sharma; Shivani Katyal; R Lucy Park; Joanne Tobacman
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2012-05-01
Journal Detail:
Title:  Pediatric pulmonology     Volume:  48     ISSN:  1099-0496     ISO Abbreviation:  Pediatr. Pulmonol.     Publication Date:  2013 Mar 
Date Detail:
Created Date:  2013-02-18     Completed Date:  2013-08-09     Revised Date:  2014-03-12    
Medline Journal Info:
Nlm Unique ID:  8510590     Medline TA:  Pediatr Pulmonol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  236-44     Citation Subset:  IM    
Copyright Information:
Copyright © 2012 Wiley Periodicals, Inc.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Asthma / blood,  enzymology
Biological Markers / blood
Case-Control Studies
Child
Child, Preschool
Cystic Fibrosis / blood,  enzymology*
Enzyme-Linked Immunosorbent Assay
Female
Humans
Interleukin-6 / blood
Leukocytes, Mononuclear / enzymology*
Male
N-Acetylgalactosamine-4-Sulfatase / blood*
Neutrophils / enzymology*
Single-Blind Method
Grant Support
ID/Acronym/Agency:
UL1 RR029879/RR/NCRR NIH HHS
Chemical
Reg. No./Substance:
0/Biological Markers; 0/Interleukin-6; EC 3.1.6.12/N-Acetylgalactosamine-4-Sulfatase; EC 3.1.6.12./arylsulfatase B, human
Comments/Corrections

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