ABNORMAL EXPRESSION OF HEPARAN-SULFATE PROTEOGLYCAN ON BASAL LAMINA OF MUSCLE-FIBERS IN 2 JAPANESE PATIENTS WITH ADHALIN DEFICIENCY

被引：11

作者：

HIGUCHI, I ^{[1
]}

FUKUNAGA, H ^{[1
]}

MATSUMURA, K ^{[1
]}

INOSE, M ^{[1
]}

IZUMI, K ^{[1
]}

OKUBO, R ^{[1
]}

NAKAGAWA, M ^{[1
]}

SHIMIZU, T ^{[1
]}

OSAME, M ^{[1
]}

机构：

[1] TEIKYO UNIV, SCH MED, DEPT NEUROL, TEIKYO, JAPAN

来源：

NEUROMUSCULAR DISORDERS | 1995年 / 5卷 / 06期

关键词：

HEPARAN SULFATE PROTEOGLYCAN; BASAL LAMINA; ADHALIN DEFICIENCY; EXTRACELLULAR MATRIX; LAMININ;

D O I：

10.1016/0960-8966(95)00010-K

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

We recently reported the selective reduction of the B1 subunit of laminin in two Japanese patients with adhalin deficiency. We here investigated immunohistochemically the expression of other components of the extracellular matrix (ECM), including collagen type IV, heparan sulfate proteoglycan (HSPG), chondroitin-4-sulfate proteoglycan, decorin, and fibronectin in adhalin deficiency, compared with other types of muscular dystrophy. We found a reduction of HSPG on the basal lamina surrounding each muscle fiber in adhalin deficiency compared with HSPG in other diseases. This finding may be characteristic evidence of the disturbance of the sarcolemma-ECM interaction and the sarcolemmal instability in adhalin deficiency. Recently, a direct role of HSPG in fibroblast growth factor (FGF) signal transduction was demonstrated. Further investigation is required to determine if the dysfunction of FGF is relevant to the pathogenesis of adhalin deficiency.

引用

页码：467 / 474

页数：8

共 26 条

[1] DISTINCTIVE PATTERNS OF BASIC FIBROBLAST GROWTH-FACTOR (BFGF) DISTRIBUTION IN DEGENERATING AND REGENERATING AREAS OF DYSTROPHIC (MDX) STRIATED MUSCLES [J].