Ikezoe K, Ohshima S, Osoegawa M, Tanaka M, Ogawa K, Nagata K, Kira J-i
Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka 812-8582, Japan.
J Neurol Neurosurg Psychiatry. 2006 Oct;77(10):1187-90. doi: 10.1136/jnnp.2005.081810.
Granulysin, a recently defined cytolytic molecule, is expressed in cytotoxic T cells and natural killer cells in a similar way to perforin, which is reported to have a major role in the pathogenesis of polymyositis and inclusion-body myositis (IBM).
To clarify the role of granulysin in polymyositis and IBM.
The expression of granulysin and perforin was examined by double staining with CD8, CD4 and CD56 in endomysial infiltrating cells and autoinvasive cells in muscle biopsy specimens of 17 patients with polymyositis (6 steroid resistant and 11 steroid responsive) and of 7 patients with IBM.
Similar to perforin, granulysin was expressed in CD8, CD4 or CD56 cells in patients with polymyositis and IBM. The ratio of cells double positive for granulysin and CD8 to all CD8 cells at endomysial sites was notably higher in steroid-resistant polymyositis than in steroid-responsive polymyositis and IBM.
Granulysin expression in CD8 cells seems to be correlated with steroid resistance in polymyositis.
颗粒溶素是一种最近确定的溶细胞分子，在细胞毒性T细胞和自然杀伤细胞中的表达方式与穿孔素相似，据报道穿孔素在多发性肌炎和包涵体肌炎（IBM）的发病机制中起主要作用。
阐明颗粒溶素在多发性肌炎和IBM中的作用。
采用CD8、CD4和CD56双重染色法，检测17例多发性肌炎患者（6例激素抵抗型和11例激素敏感型）和7例IBM患者肌肉活检标本中肌内膜浸润细胞和自身侵袭细胞中颗粒溶素和穿孔素的表达。
与穿孔素相似，颗粒溶素在多发性肌炎和IBM患者的CD8、CD4或CD56细胞中表达。在肌内膜部位，激素抵抗型多发性肌炎中颗粒溶素和CD8双阳性细胞占所有CD8细胞的比例显著高于激素敏感型多发性肌炎和IBM。
CD8细胞中颗粒溶素的表达似乎与多发性肌炎中的激素抵抗相关。
