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Correction of leukocyte adhesion deficiency type II with oral fucose.

Abstract:

We describe a simple, noninvasive, and effective therapy for leukocyte adhesion deficiency type II (LAD II), a rare inherited disorder of fucose metabolism. This disorder leads to an immunodeficiency caused by the absence of carbohydrate-based selectin ligands on the surface of neutrophils as well as to severe psychomotor and mental retardation. The fucosylation defect in LAD II fibroblasts can be corrected by addition of L-fucose to the culture medium. This prompted us to initiate dietary fu...

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Publication status:
Published

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Authors


Marquardt, T More by this author
Srikrishna, G More by this author
Freeze, HH More by this author
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Journal:
Blood
Volume:
94
Issue:
12
Pages:
3976-3985
Publication date:
1999-12-05
EISSN:
1528-0020
ISSN:
0006-4971
URN:
uuid:b6d1576c-8323-40e6-ab16-18576e489d5b
Source identifiers:
31584
Local pid:
pubs:31584

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