Murphy Charles L, Kestler Daniel P, Foster James S, Wang Shuching, Macy Sallie D, Kennel Stephen J, Carlson Eric R, Hudson John, Weiss Deborah T, Solomon Alan
Human Immunology and Cancer Program, Department of Medicine, University of Tennessee Graduate School of Medicine, Knoxville, Tennessee 37920, USA.
Amyloid. 2008 Jun;15(2):89-95. doi: 10.1080/13506120802005965.
We have previously reported that the amyloid found in three patients with calcifying epithelial odontogenic tumors (CEOT) was composed of N-terminal fragments of a putative 153-residue protein specified by a gene designated FLJ20513 now known to represent exons 5 through 10 of the odontogenic ameloblast-associated protein (ODAM) locus that encodes a 279-residue polypeptide. Confirmation of the amyloidogenic potential of ODAM has resulted from analyses of four other cases where we found, in addition, a 74-residue segment specified by exon 4. Through preparation of ODAM-related synthetic peptides, it was possible to localize the fibril-forming region of this molecule, as well as generate a monoclonal antibody that reacted specifically with the amyloid associated with CEOT. Notably, we also detected green birefringent congophilic material in unerupted tooth follicles - a precursor of CEOT - and demonstrated through immunologic and chemical analyses the ODAM nature of the deposits. Our studies have provided further evidence for this unique form of odontogenic amyloid that we provisionally designate "AODAM".
我们之前报道过，在三名患有钙化上皮性牙源性肿瘤（CEOT）的患者中发现的淀粉样蛋白由一种假定的153个残基的蛋白质的N端片段组成，该蛋白质由一个名为FLJ20513的基因指定，现在已知该基因代表牙源性成釉细胞相关蛋白（ODAM）基因座的第5至10外显子，该基因座编码一种279个残基的多肽。对另外四个病例的分析证实了ODAM的淀粉样变性潜力，在这些病例中，我们还发现了由第4外显子指定的一个74个残基的片段。通过制备与ODAM相关的合成肽，有可能定位该分子的纤维形成区域，并产生一种与CEOT相关淀粉样蛋白特异性反应的单克隆抗体。值得注意的是，我们还在未萌出的牙滤泡（CEOT的前体）中检测到绿色双折射嗜刚果红物质，并通过免疫和化学分析证明了沉积物的ODAM性质。我们的研究为这种独特形式的牙源性淀粉样蛋白提供了进一步的证据，我们暂时将其命名为“AODAM”。
