Document Detail

Oxidative stress in the brain of Fukuyama type congenital muscular dystrophy: immunohistochemical study on astrocytes.
MedLine Citation:
PMID:  12585716     Owner:  NLM     Status:  MEDLINE    
Astrocytes in the cerebrum and medulla oblongata of cases of Fukuyama type congenital muscular dystrophy were examined by immunohistochemistry of oxidative modification products and free-radical scavenging enzymes because abnormal glia limitans formed by astrocytic end feet is considered to be involved in the genesis of brain lesions of Fukuywama type congenital muscular dystrophy. The study was performed on two fetal cases of Fukuyama type congenital muscular dystrophy of 18 and 20 weeks' gestation and seven patients with Fukuyama type congenital muscular dystrophy ranging in age from 2 to 27 years. Eight age-matched control cases were used. Polymerase chain reaction (PCR) was performed to ascertain the gene phenotype of two child cases, in which prenatal gene analysis was not performed. Astrocytes, especially layer I astrocytes, of postnatal cases of Fukuyama type congenital muscular dystrophy were weakly positivefor Nepsilon-(carboxymethyl)lysine and argpyrimidine, suggesting that they were sensitive to oxidative stress, and the accumulation may be related to the abnormal glia limitans. Secondary increase of manganese (Mn) superoxide dismutase against the increase of free radicals was considered in patients with Fukuyama type congenital muscular dystrophy more than 14 years old considered to be homozygous for founder haplotype: homozygosity was suggested by PCR in two cases. In contrast, expression of Mn superoxide dismutase was decreased in 2- and 6-year-old children with Fukuyama type congenital muscular dystrophy that were heterozygous. Moreover, accumulation of argpyrimidine was exclusively found in astrocytes of the 2-year-old child that exhibited severe brain lesions. Function of astrocytes might be impaired or immature in severe or heterozygous cases. These results may confirm that astrocytes play an important role in the etiology of the brain lesion.
Tomoko Yamamoto; Noriyuki Shibata; Makio Kobayashi; Kayoko Saito; Makiko Osawa
Related Documents :
9849686 - The near complete tracheal ring deformity.
7849676 - Polymetatarsia as an etiology for tailor's bunion deformity: a surgical approach.
25064786 - Hypothermia as a clinical neuroprotectant.
7717426 - Developmental anomalies suggestive of the human homologue of the mouse mutant disorgani...
1410316 - Tipss: use of metallic stents offers non-surgical alternative.
12870116 - Persistence of a highly resistant strain of tuberculosis in new york city during 1990-1...
Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of child neurology     Volume:  17     ISSN:  0883-0738     ISO Abbreviation:  J. Child Neurol.     Publication Date:  2002 Nov 
Date Detail:
Created Date:  2003-02-14     Completed Date:  2003-05-02     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8606714     Medline TA:  J Child Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  793-9     Citation Subset:  IM    
Department of Pathology, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, Japan.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Age Factors
Astrocytes / metabolism*
Child, Preschool
Fetus / metabolism,  pathology
Medulla Oblongata / metabolism*,  pathology
Muscular Dystrophies / congenital,  metabolism*,  pathology
Oxidative Stress*
Polymerase Chain Reaction
Telencephalon / metabolism*,  pathology

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

Previous Document:  PET imaging-based evaluation of aerosol drugs and their delivery devices: nasal and pulmonary studie...
Next Document:  Epilepsy, occipital calcifications, and oligosymptomatic celiac disease in childhood.