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脯氨酰二肽酶缺乏症中溃疡形成的发病机制。血管病变和淀粉样沉积物的作用。

Pathogenesis of ulcerations in deficiency of prolidase. The role of angiopathy and of deposits of amyloid.

作者信息

Pierard G E, Cornil F, Lapiere C M

出版信息

Am J Dermatopathol. 1984 Oct;6(5):491-7. doi: 10.1097/00000372-198410000-00013.

DOI:10.1097/00000372-198410000-00013
PMID:6507815
Abstract

The structure of the skin and the pattern of alteration in chronic ulcerations associated with deficiency of prolidase have been studied superficially in the past. We examined histologically several biopsies taken from apparently normal skin and from ulcerations afflicting a young woman with such a syndrome. Deposits of amyloid were found within the walls of medium-sized vessels and occasionally occluding their lumens. Impaired cutaneous microcirculation resulting from statis, moderate vasculitis, and abnormal structure of the dermis may be responsible for the regional preponderance of deposits of amyloid and ulcerations in the legs.

摘要

过去曾对与脯氨酰肽酶缺乏相关的慢性溃疡的皮肤结构和变化模式进行过初步研究。我们对取自一名患有该综合征的年轻女性的明显正常皮肤和溃疡处的几块活检组织进行了组织学检查。在中等大小血管壁内发现了淀粉样蛋白沉积,偶尔会阻塞管腔。由于血流淤滞、中度血管炎和真皮结构异常导致的皮肤微循环受损,可能是腿部淀粉样蛋白沉积和溃疡区域优势的原因。

相似文献

1
Pathogenesis of ulcerations in deficiency of prolidase. The role of angiopathy and of deposits of amyloid.脯氨酰二肽酶缺乏症中溃疡形成的发病机制。血管病变和淀粉样沉积物的作用。
Am J Dermatopathol. 1984 Oct;6(5):491-7. doi: 10.1097/00000372-198410000-00013.
2
Chronic leg ulcerations resembling vasculitis in two siblings with prolidase deficiency.两名患有氨肽酶缺乏症的同胞兄妹出现类似血管炎的慢性腿部溃疡。
Clin Rheumatol. 1993 Sep;12(3):410-4. doi: 10.1007/BF02231590.
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An autopsy case of prolidase deficiency.一例脯氨酰肽酶缺乏症尸检病例。
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[Prolidase and manganese deficiency. Apropos of a case: diagnosis and treatment].
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Prolidase deficiency in two siblings with chronic leg ulcerations. Clinical, biochemical, and morphologic aspects.
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Autosomal recessive prolidase deficiency. Three patients with recalcitrant ulcers.常染色体隐性二肽基肽酶缺乏症。三名患有顽固性溃疡的患者。
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Molecules. 2023 Jan 14;28(2):851. doi: 10.3390/molecules28020851.
2
Recombinant Prolidase Activates EGFR-Dependent Cell Growth in an Experimental Model of Inflammation in HaCaT Keratinocytes. Implication for Wound Healing.重组脯氨酰寡肽酶在HaCaT角质形成细胞炎症实验模型中激活表皮生长因子受体(EGFR)依赖性细胞生长。对伤口愈合的意义。
Front Mol Biosci. 2022 Mar 30;9:876348. doi: 10.3389/fmolb.2022.876348. eCollection 2022.
3
Quantitative analysis of the natural history of prolidase deficiency: description of 17 families and systematic review of published cases.
脯氨酸肽酶缺乏症自然病史的定量分析:17 个家系的描述及已发表病例的系统回顾
Genet Med. 2021 Sep;23(9):1604-1615. doi: 10.1038/s41436-021-01200-2. Epub 2021 May 26.
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Extracellular Prolidase (PEPD) Induces Anabolic Processes through EGFR, β-integrin, and IGF-1R Signaling Pathways in an Experimental Model of Wounded Fibroblasts.细胞外脯氨酸内肽酶(PEPD)通过 EGFR、β-整合素和 IGF-1R 信号通路在创伤成纤维细胞的实验模型中诱导合成代谢过程。
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Prolidase Stimulates Proliferation and Migration through Activation of the PI3K/Akt/mTOR Signaling Pathway in Human Keratinocytes.脯氨酰寡肽酶通过激活人角质形成细胞中的PI3K/Akt/mTOR信号通路刺激增殖和迁移。
Int J Mol Sci. 2020 Dec 3;21(23):9243. doi: 10.3390/ijms21239243.