Department of Neurology, University of Texas Medical Branch, George and Cynthia Mitchell Center for Neurodegenerative Diseases, Galveston, TX 77555, USA.
Exp Dermatol. 2010 Oct;19(10):904-11. doi: 10.1111/j.1600-0625.2010.01121.x.
In the skin, amyloidosis can be found with or without systemic disease. Primary cutaneous amyloidosis defines those amyloidoses restricted to the skin without involvement of other systems. Here, we used conformation-specific antibodies to characterise both fibrillar and oligomeric amyloid aggregates in the skin from patients with cutaneous amyloidosis. Localised cutaneous amyloidosis with different morphology was reproduced in mice by intra-dermal (i.d.) and subdermal administration of amyloid-enhancing factor. Moreover, we demonstrated that conformational antibodies were effective in clearing amyloid deposits caused by localised intra-lesional injections without the necessity of an immune response. Given the accessibility and amyloid localization in this disease, direct i.d. injections of conformational antibodies could be a convenient and direct method for treatment.
在皮肤中,淀粉样变性可伴有或不伴有全身性疾病。原发性皮肤淀粉样变性定义为那些局限于皮肤而不涉及其他系统的淀粉样变性。在这里,我们使用构象特异性抗体来描述皮肤淀粉样变性患者皮肤中的纤维状和寡聚体淀粉样聚集物。通过皮内(i.d.)和皮下给予淀粉样增强因子,在小鼠中重现了具有不同形态的局限性皮肤淀粉样变性。此外,我们证明构象抗体可有效清除由局部病灶内注射引起的淀粉样沉积物,而无需免疫反应。鉴于这种疾病的可及性和淀粉样蛋白的定位,直接皮内注射构象抗体可能是一种方便和直接的治疗方法。