Molecular Biology for Genetic Deficiencies of Complement Components in Rabbits : C8α-γDeficiency and C3 Hypocomplementemia

JARQ : Japan Agricultural Research Quarterly
ISSN 00213551
書誌レコードID(総合目録DB) AA0068709X

The complement system is composed of approximately 20 plasma proteins and constitutes a major humoral defense and clearance system in the bloodstream. New complement deficiencies, i.e. C8α-γ deficiency (C8α-γD) and C3 hypocomplementemia (C3-hypo), were identified in rabbits. The C8α-γD and C3-hypo were transmitted as a simple autosomal recessive and condominant trait, respectively. The physiological characteristics and molecular bases for C8α-γD and C3-hypo were identified as follows: C8α-γD is characterized by dwarfism (non-pituitary), small litter size, small thymus, a low survival rate, severely reduced serum bactericidal activity and enhanced delayed-type hypersensitivity (DTH), and normal expression of α and γ genes, but abnormal co-translational processing of C8α gene (a mutation of the exon/intron junction of the C8α gene). C3-hypo is characterized by approximately 10% of normal serum C3 levels, a low survival rate, reduced serum bactericidal activity, suppressed DTH, and low levels of liver C3 mRNA (pretranslational defect), and C3 phenotypes are dependent on RFLPs of the C3 gene.

作成者 Masanori KOMATSU
国立情報学研究所メタデータ主題語彙集(資源タイプ) Journal Article
開始ページ 48
終了ページ 54
言語 eng