1995 Fiscal Year Final Research Report Summary
Studies on causative gene defects in primary neutrophil abnormalities with a purpose of applying it to the gene therapy
| Project/Area Number |
06454299
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| Research Category |
Grant-in-Aid for General Scientific Research (B)
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| Allocation Type | Single-year Grants |
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| Research Field |
Pediatrics
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| Research Institution | Shinshu University |
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Principal Investigator |
KOMIYAMA Atsushi Shinshu Univ. School Med., Dept. Pediatr., Prof., 医学部, 教授 (50020798)
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| Co-Investigator(Kenkyū-buntansha) |
YASUI Kozo Shinshu Univ. School Med. Hosp., Dept. Pediatr., Assistant Prof., 医学部附属病院, 講師 (90200493)
KOIKE Kenichi Shinshu Univ. School Med., Dept. Pediatr., Associate Prof., 医学部, 助教授 (40143979)
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| Project Period (FY) |
1994 – 1995
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| Keywords | neutrophil / neutrophil functtion / immunodeficiency / neutropenia / neutrophil dysfunction / neutrophil-specific granule deficiency / G-CSF receptor / bactericidal proteins |
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| Research Abstract |
The present studies were conducted to analyze the induction of neutrophil functions and its signal transduction and to determine the causative gene defects in primary neutrophil abnormalities with a purpose of applying it to the gene therapy.
1. Analysis of induction of neutrophil functions and its signal transduction
(1) Neutrophil functions were induced via CD11b, an adhesion molecule, which plays a role in regulating the functions by the interaction between neutrophils and endothelial cells.
(2) The tyrosine kinase was demonstrated to be a specific signaling pathway for FMLP-induced chemotaxis.
2. Analysis of causative gene defects in primary neutrophil abnormalities
(1) Congenital neutropenia (kostmann type) : It was possible to get neutrophils enough to use for the present studies by culturing the bone marrow cells in two patients. The G-CSF receptor was not decreased in the number, and its mRNA expression was normal.
(2) Congenital neutrophil-specific granule deficiency : There was no or reduced expression of mRNA for not only defensins but also lactoferrin and collagenase. Southern blot analysis of these genomic DNAs failed to demonstrate abnormal band by use of several restriction enzymes. Studies are now in progress to analyze the flanking regions and promoter of the genes.
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Research Products
(18 results)
