lonza,CC-7231,DHLF-IPF-患病的人肺成纤维细胞特发性肺纤维化

Product Overview

IPF (Idiopathic Pulmonary Fibrosis) is a fatal disease that usually affects adults between the ages of 50-70. The disease is characterized by progressive decline in lung function resulting from scarring of lung tissue.

IPF varies from person to person where in some, fibrosis happens quickly, while the process can be much slower in others. IPF has no cure yet and both geneticand environmental factors are attributed to the development of the disease. Genetics may play a role in causing IPF, and cigarette smoking is the most accepted risk factor in increasing the chances of developing IPF. Recent research has helped doctors understand the disease more closely so they can more quickly diagnose it than in the past. Efforts are still underway in the research community to understand the pathways of disease progression and subsequently develop cures for IPF. IPF was once thought to be a chronic inflammatory process but recent evidence suggests that the abnormal activation of alveolar epithelial cells (AECs) induce over-activation of fibrotic response. The fibroblast and myofibroblast foci secrete excessive amounts of collagens, leading to scarringand destruction of the lung architecture. The mechanisms that link IPF with ageing and aberrant epithelial activation are unknown.

To understand the progression of IPF disease, Lonza offers cryopreserved lung fibroblasts isolated from donors diagnosed with IPF. Cryopreserved IPF fibroblasts are shipped at passage 2 and stain negative for von Willebrand factor VIII, cytokeratins 14, 18 and 19, and alpha smooth muscle actin. All cells test negative for mycoplasma, bacteria, yeast, and fungi. HIV-1, hepatitis B and hepatitis C are not detected for all donors and/or cell lots. A Certificate of Analysis is provided for each cell lot purchased.

IPF（特发性肺纤维化）是一种致命疾病，通常影响 50-70 岁的成年人。该疾病的特征是肺组织瘢痕形成导致肺功能进行性下降。

IPF 因人而异，在某些人中，纤维化发生得很快，而在另一些人中该过程可能要慢得多。 IPF 还没有治愈方法，遗传和环境因素都归因于疾病的发展。遗传可能在导致 IPF 中起作用，而吸烟是增加 IPF 发生机会的最公认的风险因素。最近的研究帮助医生更深入地了解这种疾病，因此他们可以比过去更快地诊断它。研究界仍在努力了解疾病进展的途径并随后开发 IPF 的治疗方法。 IPF 曾被认为是一种慢性炎症过程，但最近的证据表明，肺泡上皮细胞 (AEC) 的异常激活会导致纤维化反应的过度激活。成纤维细胞和肌成纤维细胞病灶分泌过量的胶原蛋白，导致肺结构的瘢痕形成和破坏。将 IPF 与衰老和异常上皮激活联系起来的机制尚不清楚。

为了了解 IPF 疾病的进展，Lonza 提供了从诊断为 IPF 的供体中分离出来的冷冻保存的肺成纤维细胞。冷冻保存的 IPF 成纤维细胞在第 2 代运输，并且对血管性血友病因子 VIII、细胞角蛋白 14、18 和 19 以及 α 平滑肌肌动蛋白染色呈阴性。所有细胞的支原体、细菌、酵母和真菌均呈阴性。并非所有供体和/或细胞批次都检测到 HIV-1、乙型肝炎和丙型肝炎。为购买的每个细胞批次提供分析证书。

Relevant Downloads (Lonza’s Normal Human Lung Fibroblasts for IPF Research):

• Lung Epithelium Overexpressed Noncoding RNA (LEON): Role of a Novel Long Intergenic Noncoding RNA in Idiopathic Pulmonary Fibrosis
• The IL-33/ST2 Axis Is Upregulated in Fibrotic Lung Disease
• Extracellular Matrix Metalloproteinase Inducer (EMMPRIN) promotes lung fibroblast proliferation, survival and differentiation to myofibroblasts