Heterogeneity of Fibroblasts and Myofibroblasts in Pulmonary Fibrosis.

Q1 Medicine

Current Pathobiology Reports Pub Date : 2017-06-01 Epub Date: 2017-05-02 DOI:10.1007/s40139-017-0134-x

David M Habiel, Cory M Hogaboam

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Abstract

Purpose of review: Idiopathic Pulmonary Fibrosis (IPF) is the most common form of interstitial lung diseases of unknown eathiopathogenesis, mean survival of 3-5 years and limited therapeutics. Characterized by a loss of alveolar type II epithelial cells and aberrant activation of stromal cells, considerable effort was undertaken to characterize the origin and activation mechanisms of fibroblasts and myofibroblasts in IPF lungs. In this review, the origin and contribution of fibroblast and myofibroblasts in lung fibrosis will be summarized.

Recent findings: Lineage tracing experiments suggested that interstitial lung fibroblasts and lipofibroblasts, pericytes and mesothelial cells differentiate into myofibroblasts. However, epithelial and bone marrow derived cells may give rise to collagen expressing fibroblasts but do not differentiate into myofibroblasts.

Summary: There is great heterogeneity in fibroblasts and myofibroblasts in fibrotic lungs. Further, there is evidence for the expansion of pericyte derived myofibroblasts and loss of lipofibroblasts and lipofibroblast derived myofibroblasts in IPF.

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肺纤维化中成纤维细胞和肌成纤维细胞的异质性

综述的目的：特发性肺纤维化（IPF）是间质性肺病中最常见的一种，发病机制不明，平均存活期为 3-5 年，治疗手段有限。其特点是肺泡 II 型上皮细胞缺失和基质细胞异常活化，因此，人们花费了大量精力研究 IPF 肺中成纤维细胞和肌成纤维细胞的起源和活化机制。本综述将总结成纤维细胞和肌成纤维细胞在肺纤维化中的起源和贡献：系谱追踪实验表明，肺间质成纤维细胞和脂成纤维细胞、周细胞和间皮细胞分化为肌成纤维细胞。然而，上皮细胞和骨髓衍生细胞可能产生表达胶原蛋白的成纤维细胞，但不会分化成肌成纤维细胞。此外，有证据表明，在 IPF 中，周细胞衍生的肌成纤维细胞扩大，而脂肪成纤维细胞和脂肪成纤维细胞衍生的肌成纤维细胞消失。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Current Pathobiology Reports Medicine-Pathology and Forensic Medicine

CiteScore

6.40

自引率

0.00%

发文量

期刊介绍： This journal aims to offer expert review articles on the most important recent research pertaining to biological mechanisms underlying disease, including etiology, pathogenesis, and the clinical manifestations of cellular alteration. By providing clear, insightful, balanced contributions, the journal intends to serve those for whom the elucidation of new techniques and technologies related to pathobiology is essential. We accomplish this aim by appointing international authorities to serve as Section Editors in key subject areas across the field. Section Editors select topics for which leading experts contribute comprehensive review articles that emphasize new developments and recently published papers of major importance, highlighted by annotated reference lists. An Editorial Board of more than 20 internationally diverse members reviews the annual table of contents, ensures that topics include emerging research, and suggests topics of special importance to their country/region. Topics covered may include autophagy, cancer stem cells, induced pluripotential stem cells (iPS cells), inflammation and cancer, matrix pathobiology, miRNA in pathobiology, mitochondrial dysfunction/diseases, and myofibroblast.