|
|
|
|
| LEADER |
01000naa a22002652 4500 |
| 001 |
NLM156560453 |
| 003 |
DE-627 |
| 005 |
20231223074414.0 |
| 007 |
tu |
| 008 |
231223s2005 xx ||||| 00| ||eng c |
| 028 |
5 |
2 |
|a pubmed24n0522.xml
|
| 035 |
|
|
|a (DE-627)NLM156560453
|
| 035 |
|
|
|a (NLM)16019263
|
| 040 |
|
|
|a DE-627
|b ger
|c DE-627
|e rakwb
|
| 041 |
|
|
|a eng
|
| 100 |
1 |
|
|a Yan, Sen Rong
|e verfasserin
|4 aut
|
| 245 |
1 |
0 |
|a Increased chemoattractant induced neutrophil oxidative burst, accelerated apoptosis, and dysregulated tyrosine phosphorylation associated with lifelong bacterial infections
|
| 264 |
|
1 |
|c 2005
|
| 336 |
|
|
|a Text
|b txt
|2 rdacontent
|
| 337 |
|
|
|a ohne Hilfsmittel zu benutzen
|b n
|2 rdamedia
|
| 338 |
|
|
|a Band
|b nc
|2 rdacarrier
|
| 500 |
|
|
|a Date Completed 27.10.2005
|
| 500 |
|
|
|a Date Revised 21.11.2013
|
| 500 |
|
|
|a published: Print
|
| 500 |
|
|
|a Citation Status MEDLINE
|
| 520 |
|
|
|a A boy with lifelong recurrent bacterial infection at cutaneous and mucosal sites was investigated. PMN oxidative burst to phorbol myristate acetate (PMA) and zymosan was normal but was increased 20- to 50-fold upon C5a or formyl-met-leu-phe (fMLP) chemoattractant stimulation, accompanied by accelerated PMN apoptosis. His PMNs showed increased constitutive tyrosine phosphorylation of 21-, 25-, and 44-kDa proteins, and of src-family kinases (p59(hck), p58(fgr), and p53/56(lyn)). Phosphorylation was abnormally enhanced following fMLP stimulation. Expression and activity of the major PMN tyrosine phosphatases, i.e., CD45, CD148, and SHP-1 and -2, was normal. However, dephosphorylation of phospho-p58(fgr) and phospho-p53/56(lyn) by lysates of patient's PMNs was enhanced. Thus, another phosphatase may be overactive, perhaps dephosphorylating a regulatory (inhibitory) site on a protein tyrosine kinase, accounting for the abnormal PMN tyrosine phosphorylation and function. With age (now 13 years), T-cell lymphopenia and loss of T-cell responses developed. This appears to be a unique primary immunodeficiency with abnormal PMN oxidative and apoptotic responses to chemoattractants, dysregulated protein tyrosine phosphorylation, serious bacterial infection, and T-lymphocyte attrition
|
| 650 |
|
4 |
|a Case Reports
|
| 650 |
|
4 |
|a Journal Article
|
| 650 |
|
4 |
|a Research Support, Non-U.S. Gov't
|
| 650 |
|
7 |
|a Chemotactic Factors
|2 NLM
|
| 650 |
|
7 |
|a Tyrosine
|2 NLM
|
| 650 |
|
7 |
|a 42HK56048U
|2 NLM
|
| 700 |
1 |
|
|a Bortolussi, Robert
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Issekutz, Thomas B
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Issekutz, Andrew C
|e verfasserin
|4 aut
|
| 773 |
0 |
8 |
|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 117(2005), 1 vom: 15. Okt., Seite 36-47
|w (DE-627)NLM098196855
|x 1521-7035
|7 nnns
|
| 773 |
1 |
8 |
|g volume:117
|g year:2005
|g number:1
|g day:15
|g month:10
|g pages:36-47
|
| 912 |
|
|
|a GBV_USEFLAG_A
|
| 912 |
|
|
|a SYSFLAG_A
|
| 912 |
|
|
|a GBV_NLM
|
| 912 |
|
|
|a GBV_ILN_11
|
| 912 |
|
|
|a GBV_ILN_24
|
| 912 |
|
|
|a GBV_ILN_350
|
| 951 |
|
|
|a AR
|
| 952 |
|
|
|d 117
|j 2005
|e 1
|b 15
|c 10
|h 36-47
|