Pantetheinase Activity and Cysteamine Content in Cystinotic and Normal Fibroblasts and Leukocytes
作者:
SHELDON ORLOFF,
JEAN BUTLER,
DAVID TOWNE,
ANIL MUKHERJEE,
JOSEPH SCHULMAN,
期刊:
Pediatric Research
(OVID Available online 1981)
卷期:
Volume 15,
issue 7
页码: 1063-1067
ISSN:0031-3998
年代: 1981
出版商: OVID
关键词: cysteamine;leukocyte;cystinosis;pantetheinase;fibroblast
数据来源: OVID
摘要:
SummaryCysteamine is the most effective agent known for the reduction of the elevated cystine content of cells from patients with cystinosis. A defect in endogenous cysteamine generation could account for many of the metabolic features of this disorder. To test this hypothesis, we have developed improved methods for measuring pantetheinase (cysteamine-generating) activity and intracellular cysteamine levels and used these methods to measure such parameters in cystinotic and normal leukocytes and cultured skin fibroblasts. Pantetheinase activity as defined in the text was similar in extracts of cystinotic and normal cells [leucocytes, normal, 78 ± 15 (S.E.), cystinotic, 56 ± 6.4; fibroblasts, normal, 9.4 ± 1.5; cystinotic, 7.7 ± 1.7]. Cysteamine levels were normal in leukocytes from cystinotics receiving no cysteamine or doses of oral cysteamine too low to reduce leukocyte cystine content. The results indicate that the cause of cystinosis is unlikely to be related to a failure to generate or sustain normal intracellular cysteamine levels.
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