Novel histopathological deposition patterns of EGF-containing fibulin-like extracellular matrix protein 1 amyloidosis: an autopsy case exhibiting a possible association between AEFEMP1 amyloidosis and elastic fibres
Novel histopathological deposition patterns of EGF-containing fibulin-like extracellular matrix protein 1 amyloidosis: an autopsy case exhibiting a possible association between AEFEMP1 amyloidosis and elastic fibres
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含有 EGF 的纤维蛋白样细胞外基质蛋白 1 淀粉样变性的新型组织病理学沉积模式：尸检病例显示 AEFEMP1 淀粉样变性与弹性纤维之间可能存在关联
DOI：
10.1080/13506129.2021.2020754
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发表时间：
2022
期刊：
影响因子：
5.5
通讯作者：
Nishida Naoki
中科院分区：
文献类型：
--
作者：
Ichimata Shojiro;Hata Yukiko;Katoh Nagaaki;Kametani Fuyuki;Yazaki Masahide;Sekijima Yoshiki;Nishida Naoki
含EGF的纤维蛋白样细胞外基质蛋白1淀粉样蛋白（AEFEMP 1）是一种新发现的淀粉样纤维蛋白[1-3];其沉积通常是偶然发现，其病理学意义尚不清楚[2，3]。虽然AEFEMP 1沉积被归类为门静脉上的局部淀粉样变性，但在下胃肠道以外的血管上观察到其沉积[2]。因此，AEFEMP 1淀粉样变性可能影响全身器官。然而，没有报告评估其在除血管外的其他组织中的全身组织病理学沉积模式。患者有高血压病史，但未报告明显腹部症状。法医尸检证实了双肺的组织学发现，表明是溺水。在血管上偶然发现AEFEMP 1沉积物（图1（ac））。在回肠中，还在肌间神经丛周围和浆膜中鉴定到AEFEMP 1沉积物（图1（d-f））。此外，在中小血管和肠系膜血管中发现淀粉样蛋白沉积（图1（g-i））。后一种沉积物显示出具有弹性纤维芯的特征性珠状图案（图1（g））。如前所述[4]，使用激光显微切割-液相色谱-串联质谱法进行蛋白质组学分析，证实存在AEFEMP 1沉积物（图1（j）和补充图1）。还鉴定了主要的淀粉样纤维相关蛋白（图1（j））。在结肠中也观察到类似的沉积模式（补充图2）。在回肠和结肠中，肌间神经丛中的神经元计数中度减少。此外，肌间神经丛周围的Cajal间质细胞几乎完全消失（补充图3）。免疫组织化学结果的详细信息总结见补充表1。我们检测了AEFEMP 1在全身器官和组织中的分布。结果总结见补充表2。简而言之，EFEMP 1表达在全身小至中等大小血管的壁上、胸膜中、胃肠道的浆膜上、骨骼肌的平滑肌和脂肪组织中鉴定。同时，在肺、胃肠道、骨骼肌和胃肠道中均可见淀粉样蛋白沉积，
EGF-containing fibulin-like extracellular matrix protein 1 amyloid (AEFEMP1) is a newly recognised amyloid fibril protein [1-3]; its deposition is usually an incidental finding and its pathological significance is unknown [2, 3]. Although AEFEMP1 deposition is categorised as localised amyloidosis on the portal veins, its deposition has been observed on vessels other than those in the lower gastrointestinal tract [2]. Therefore, AEFEMP1 amyloidosis possibly affects systemic organs. However, no reports have evaluated its systemic histopathological deposition patterns in other tissues apart from blood vessels.An 81-year-old man was found dead in an aqueduct. He had a medical history of hypertension, but no complaints of remarkable abdominal symptoms were reported. The forensic autopsy confirmed histological findings in both lungs, which indicated drowning. AEFEMP1 deposits were incidentally found on the vessels (Figure 1 (ac)). In the ileum, AEFEMP1 deposits were also identified around the myenteric plexus and in the serosa (Figure 1 (d-f)). Furthermore, amyloid deposition was identified in the medium to small vessels and in the interstitium of the mesentery (Figure 1 (g-i)). The latter deposits exhibited a characteristic bead-like pattern with an elastic fibre core (Figure 1 (g)). Proteomic analysis using laser microdissection-liquid chromatography-tandem mass spectrometry was performed as previously described [4], which confirmed the presence of AEFEMP1 deposits (Figure 1 (j) and Supplementary Figure 1). Major amyloid fibril-associated proteins were also identified (Figure 1 (j)). Similar deposition patterns were also observed in the colon (Supplementary Figure 2). In the ileum and colon, neuron counts were moderately decreased in the myenteric plexus. Furthermore, interstitial cells of Cajal around the circumference of the myenteric plexus were almost completely eliminated (Supplementary Figure 3). Details on the immunohistochemistry findings are summarised in Supplementary Table 1. We examined the distribution of AEFEMP1 in systemic organs and tissues. The results are summarised in Supplementary Table 2. Briefly, EFEMP1 expression was identified on the walls of systemic small-to-medium-sized vessels, in the pleura, on the serosa of the gastrointestinal tract, in the interstitium of the skeletal muscle, and adipose tissue. Meanwhile, amyloid deposition was recognisable in the lungs, gastrointestinal tract, interstitium of skeletal
影响因子：
3.5
作者：
McLaughlin, Precious J.;Bakall, Benjamin;Marmorstein, Lihua Y.
通讯作者：
Marmorstein, Lihua Y.
DOI：
10.2169/internalmedicine.5126-20
发表时间：
2021-02-15
期刊：
Internal medicine (Tokyo, Japan)
影响因子：
--
作者：
Yoshinaga T;Katoh N;Yazaki M;Sato M;Kametani F;Yasuda H;Watanabe K;Kawata K;Nakagawa M;Sekijima Y
通讯作者：
Sekijima Y
在手术切除的结肠癌标本中偶然发现含有 EGF 的纤维蛋白样细胞外基质蛋白 1 淀粉样蛋白：强调未识别类型的病例报告
影响因子：
5.5
作者：
Tsukamoto Yoshitane;Tasaki Masayoshi;Fujii Hitoshi;Tsujie Masaki;Ueda Mitsuharu
通讯作者：
Ueda Mitsuharu