Partial correction of neutrophil dysfunction by oral galactose therapy in glycogen storage disease type Ib

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• 作者：Rudolf Letkemann^a ; ^{r.letkemann@uni-muenster.de} ; Helmut Wittkowski^b ; ^{helmut.wittkowski@ukmuenster.de} ; Aristotelis Antonopoulos^c ; ^{a.antonopoulos@imperial.ac.uk} ; Teodor Podskabi^d ; ^{metabolic-genetic-disease@gmx.de} ; Stuart M. Haslam^c ; ^{s.haslam@imperial.ac.uk} ; Dirk Fö ; ll^b ; ^{dfoell@uni-muenster.de} ; Anne Dell^c ; ^{a.dell@imperial.ac.uk} ; Thorsten Marquardt^a ; ^{marquat@uni-muenster.de}
• 关键词：CHAPS ; 3-[(3-cholamidopropyl)dimethylammonio]-1-propanesulfonate ; CDG ; congenital disorder of glycosylation ; DHR ; dihydrorhodamine ; E. coli ; Escherichia coli ; EDTA ; ethylenediaminetetraacetic acid ; FICT ; fluorescein isothiocyanate ; fMLP ; formyl-methionyl-leucyl-phenylalanine ; G1P ; glucose 1-phosphate ; G6P ; glucose 6-phosphate ; G6PC ; glucose 6-phosphate catabolic ; G6PT ; glucose 6-phosphate transporter ; GCSF ; granulocyte-colony stimulating factor ; GSD-Ib ; glycogen storage disease type Ib ; HMPS ; he
• 刊名：International Immunopharmacology
• 出版年：2017
• 出版时间：March 2017
• 年：2017
• 卷：44
• 期：Complete
• 页码：216-225
• 全文大小：1553 K
• 卷排序：44

文摘

Dietary galactose led to partial improvement of respiratory burst activity. No lasting effect on hypoglycosylation of GSD-Ib neutrophils by oral galactose Impaired migratory activity of GSD-Ib neutrophils could not be restored by galactose therapy.