Document Detail


Genetic insights into congenital neutropenia.
MedLine Citation:
PMID:  19440858     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Congenital neutropenia syndromes comprise a heterogeneous group of disorders leading to increased susceptibility to bacterial infections. Recent work has elucidated the molecular basis of several congenital neutropenia syndromes such as mutations in ELA2, HAX1, GF11, and WAS. In addition, a number of complex clinical syndromes associating congenital neutropenia have been recognized and elucidated on a genetic level, e.g. p14-deficiency or G6PC3-deficiency. The clinical and genetic findings of various neutropenia syndromes are being discussed.
Authors:
Christoph Klein; Karl Welte
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Clinical reviews in allergy & immunology     Volume:  38     ISSN:  1559-0267     ISO Abbreviation:  Clin Rev Allergy Immunol     Publication Date:  2010 Feb 
Date Detail:
Created Date:  2010-01-25     Completed Date:  2010-04-02     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9504368     Medline TA:  Clin Rev Allergy Immunol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  68-74     Citation Subset:  IM    
Affiliation:
Department of Pediatric Hematology/Oncology, Hannover Medical School, Hannover, Germany. klein.christoph@mh-hannover.de
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MeSH Terms
Descriptor/Qualifier:
DNA-Binding Proteins / genetics
Glucose-6-Phosphatase / genetics
Humans
Immunologic Deficiency Syndromes / congenital*,  genetics*
Leukocyte Elastase / genetics
Mutation
Neutropenia / complications,  congenital*,  genetics*
Proteins / genetics
Transcription Factors / genetics
Chemical
Reg. No./Substance:
0/DNA-Binding Proteins; 0/GFI1 protein, human; 0/HAX1 protein, human; 0/Proteins; 0/Transcription Factors; EC 3.1.3.9/Glucose-6-Phosphatase; EC 3.1.3.9./G6PC3 protein, human; EC 3.4.21.37/Leukocyte Elastase

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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