中华检验医学杂志
中華檢驗醫學雜誌
중화검험의학잡지
CHINESE JOURNAL OF LABORATORY MEDICINE
2012年
12期
1159-1162
,共4页
张淑芳%王顺兰%张应爱%肖敬川%黄邓高
張淑芳%王順蘭%張應愛%肖敬川%黃鄧高
장숙방%왕순란%장응애%초경천%황산고
血小板减少%血小板膜糖蛋白类%肌球蛋白重链%分子马达蛋白质类%流式细胞术
血小闆減少%血小闆膜糖蛋白類%肌毬蛋白重鏈%分子馬達蛋白質類%流式細胞術
혈소판감소%혈소판막당단백류%기구단백중련%분자마체단백질류%류식세포술
Thrombocytopenia%Platelet membrane glycoproteins%Myosin heavy chains%Molecular motor proteins%Flow cytometry
目的 分析该非肌性肌球蛋白重链9基因相关疾病(MYH9-RD)家系患者多种血小板膜糖蛋白的表达.方法 采集MYH9-RD家系中11例患者和7名健康对照者的外周血；应用流式细胞术对血小板膜糖蛋白GPⅡb/Ⅲa(CD41/61)、GP Ⅰ a(CD49b)、GPⅠb/Ⅸ/Ⅴ(CD42a),GPⅠ b(CD42b)和GPⅣ(CD36)进行检测.结果 MYH9-RD家系GPⅡb/Ⅲa(CD41/61)、GP Ⅰ b(CD42b)、GPⅣ(CD36)和GP Ⅰ b/Ⅸ/Ⅴ(CD42a)复合物表达量分别为653.7±192.7、420.0±151.3、667.7±371.3和236.4±64.2,高于对照组(分别为406.7±126.1、181.2±29.3、271.4±91.6和136.1±23.5,P＜0.01),差异具有统计学意义；MYH9-RD家系患者组GP Ⅰ a(CD49b)表达量为192.2±143.4,较对照组(139.1±54.9)稍有下降,差异无统计学意义(P＞0.05).结论 本MYH9-RD家系患者临床表型复杂多样可能与血小板膜糖蛋白表达量有关.
目的 分析該非肌性肌毬蛋白重鏈9基因相關疾病(MYH9-RD)傢繫患者多種血小闆膜糖蛋白的錶達.方法 採集MYH9-RD傢繫中11例患者和7名健康對照者的外週血；應用流式細胞術對血小闆膜糖蛋白GPⅡb/Ⅲa(CD41/61)、GP Ⅰ a(CD49b)、GPⅠb/Ⅸ/Ⅴ(CD42a),GPⅠ b(CD42b)和GPⅣ(CD36)進行檢測.結果 MYH9-RD傢繫GPⅡb/Ⅲa(CD41/61)、GP Ⅰ b(CD42b)、GPⅣ(CD36)和GP Ⅰ b/Ⅸ/Ⅴ(CD42a)複閤物錶達量分彆為653.7±192.7、420.0±151.3、667.7±371.3和236.4±64.2,高于對照組(分彆為406.7±126.1、181.2±29.3、271.4±91.6和136.1±23.5,P＜0.01),差異具有統計學意義；MYH9-RD傢繫患者組GP Ⅰ a(CD49b)錶達量為192.2±143.4,較對照組(139.1±54.9)稍有下降,差異無統計學意義(P＞0.05).結論 本MYH9-RD傢繫患者臨床錶型複雜多樣可能與血小闆膜糖蛋白錶達量有關.
목적 분석해비기성기구단백중련9기인상관질병(MYH9-RD)가계환자다충혈소판막당단백적표체.방법 채집MYH9-RD가계중11례환자화7명건강대조자적외주혈；응용류식세포술대혈소판막당단백GPⅡb/Ⅲa(CD41/61)、GP Ⅰ a(CD49b)、GPⅠb/Ⅸ/Ⅴ(CD42a),GPⅠ b(CD42b)화GPⅣ(CD36)진행검측.결과 MYH9-RD가계GPⅡb/Ⅲa(CD41/61)、GP Ⅰ b(CD42b)、GPⅣ(CD36)화GP Ⅰ b/Ⅸ/Ⅴ(CD42a)복합물표체량분별위653.7±192.7、420.0±151.3、667.7±371.3화236.4±64.2,고우대조조(분별위406.7±126.1、181.2±29.3、271.4±91.6화136.1±23.5,P＜0.01),차이구유통계학의의；MYH9-RD가계환자조GP Ⅰ a(CD49b)표체량위192.2±143.4,교대조조(139.1±54.9)초유하강,차이무통계학의의(P＞0.05).결론 본MYH9-RD가계환자림상표형복잡다양가능여혈소판막당단백표체량유관.
Objective To explore the pathogenic mechanism by detecting the expression of membrane glycoprotein in the platelets of nonmuscle myosin heavy chain 9 related disease (MYH9-RD)patients.Methods Periperal bloods were obtained from 11 MYH9-RD patients and 7 normal family members.Flow cytometry was used for detecting the expression of the platelet membrane glycoprotein including GP Ⅱ b/Ⅲa(CD41/61),GP Ⅰ a(CD49b),GP Ⅰ b/Ⅸ/Ⅴ (CD42a) GP Ⅰ b(CD42b) and GPⅣCD36).Results The expression fluorescence intensity of platelet membrane glycoprotein GP Ⅱ b/Ⅲ a CD41/61),GPⅠa(CD49b),GP Ⅰ b/Ⅸ/Ⅴ (CD42a) GP Ⅰ b(CD42b) and GPⅣ (CD36) are 653.7 ±192.7,420.0 ± 151.3,667.7 ± 371.3 and 236.4 ± 64.2 respectively,which are significantly higher than those in normal controls (406.7 ± 126.1,181.2 ± 29.3,271.4 ± 91.6 and 136.1 ± 23.5 ; P ＜ 0.01) ; The expression of GP Ⅰ a(CD49b) was lower in patients with MYH9-RD (139.1 ± 54.9) than that in normal controls (192.2 ± 143.4),but there was no significant difference (P ＞ 0.05).Conclusion In our study,the diverse clinical manifestations in patients with MYH9-RD is probably associated with the expression level of platelet membrane glycoprotein
