Ansari A A, Wang Y C, Danner D J, Gravanis M B, Mayne A, Neckelmann N, Sell K W, Herskowitz A
Department of Pathology, Emory University School of Medicine, Atlanta, Georgia 30322.
Am J Pathol. 1991 Aug;139(2):337-54.
Autoantibodies against the adenine nucleotide translocator (ANT), the branched chain alpha-ketoacid dehydrogenase (BCKD) complex proteins, and myosin have been implicated in the pathogenesis of human dilated cardiomyopathy (DCM). Cardiac tissue from patients with DCM and, for control purposes, cardiac tissue from patients with other forms of cardiomyopathy and from patients with no history of cardiac disease were stained with heterologous and ANT-, BCKD-, and myosin-specific affinity-purified sera from DCM patients. Data demonstrate that although anti-myosin stains tissues from both patients and normal controls, the ANT- and BCKD-specific heterologous and affinity-purified sera from DCM patients stain only cardiac tissues from DCM patients. Intense staining in patchy areas of cardiac tissue suggests that abnormal increased expression of these putative autoantigens occurs in discrete areas of cardiac myocytes. The reactivity of the antisera was organ specific and only seen in tissues from DCM patients. The organ and disease specificity of these findings suggests that such expression may play an important role in the pathogenesis of human DCM.
针对腺嘌呤核苷酸转位酶(ANT)、支链α-酮酸脱氢酶(BCKD)复合体蛋白以及肌球蛋白的自身抗体已被认为与人类扩张型心肌病(DCM)的发病机制有关。用来自DCM患者的异源及ANT、BCKD和肌球蛋白特异性亲和纯化血清,对DCM患者的心脏组织以及作为对照的其他形式心肌病患者和无心脏病史患者的心脏组织进行染色。数据表明,尽管抗肌球蛋白血清可对患者和正常对照的组织进行染色,但来自DCM患者的ANT和BCKD特异性异源及亲和纯化血清仅对DCM患者的心脏组织进行染色。心脏组织斑片状区域的强烈染色表明,这些假定自身抗原的异常表达增加发生在心肌细胞的离散区域。抗血清的反应具有器官特异性,仅在DCM患者的组织中可见。这些发现的器官和疾病特异性表明,这种表达可能在人类DCM的发病机制中起重要作用。