Document Detail

Late-onset acid maltase deficiency. Biochemical studies of leukocytes.
MedLine Citation:
PMID:  7014786     Owner:  NLM     Status:  MEDLINE    
The activities of acid and neutral maltase were measured in lymphocytes, granulocytes, and platelets isolated from controls and from 5 patients with late-onset acid maltase deficiency (AMD). Lymphocytes from patients had markedly decreased activity of acid maltase and elevated neutral/acid ratios. In granulocytes, acid maltase was also lower than in controls, but significant activity was retained at pH 4: neutral/acid ratios were consistently elevated. Normal platelets had low acid and high neutral maltase activities: both enzyme activities varied within wide ranges and patients could not be distinguished from controls. The variable proportion of different cell types in unfractionated leukocyte preparations may yield unreliable values when used for detection of AMD. However, lymphocytes isolated from 20 ml of blood provide a readily accessible and reliable source of tissue for accurate diagnosis.
S Shanske; S Dimauro
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Journal of the neurological sciences     Volume:  50     ISSN:  0022-510X     ISO Abbreviation:  J. Neurol. Sci.     Publication Date:  1981 Apr 
Date Detail:
Created Date:  1981-07-09     Completed Date:  1981-07-09     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0375403     Medline TA:  J Neurol Sci     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  57-62     Citation Subset:  IM    
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MeSH Terms
Glucosidases / deficiency*
Hydrogen-Ion Concentration
Leukocytes / enzymology*
Muscular Atrophy / enzymology
alpha-Glucosidases / deficiency*
Grant Support
Reg. No./Substance:
EC 3.2.1.-/Glucosidases; EC

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