丹曲林对儿茶酚胺能多形性室性心动过速患者的抗心律失常效果及iPSC模型复制反应

数据集获取（Figshare）

• 源站标题：Antiarrhythmic Effects of Dantrolene in Patients with Catecholaminergic Polymorphic Ventricular Tachycardia and Replication of the Responses Using iPSC Models
• DOI：10.1371/journal.pone.0125366
• 发布日期：2015-05-08
• 文件数：6 个
• 文件总体积：1.01 MB
• 源站页面：https://plos.figshare.com/articles/dataset/Antiarrhythmic_Effects_of_Dantrolene_in_Patients_with_Catecholaminergic_Polymorphic_Ventricular_Tachycardia_and_Replication_of_the_Responses_Using_iPSC_Models/2666056

命令行下载

curl -L -o "S1 Fig.tif" "https://ndownloader.figshare.com/files/4328173"
curl -L -o "S2 Fig.tif" "https://ndownloader.figshare.com/files/4328176"
curl -L -o "S1 Protocol.docx" "https://ndownloader.figshare.com/files/4328179"
curl -L -o "S2 Protocol.doc" "https://ndownloader.figshare.com/files/4328182"
curl -L -o "S1 Table.docx" "https://ndownloader.figshare.com/files/4328185"
curl -L -o "S2 Table.docx" "https://ndownloader.figshare.com/files/4328188"

文件清单

• S1 Fig.tif（621.72 KB）
• S2 Fig.tif（264.52 KB）
• S1 Protocol.docx（16.30 KB）
• S2 Protocol.doc（79.36 KB）
• S1 Table.docx（13.42 KB）
• S2 Table.docx（14.89 KB）

文件总体积 1.01 MB（源站 API 实测）。

数据加载示例（图像类）

from PIL import Image
import glob, os

files = (glob.glob(os.path.join(path, "**", "*.png"), recursive=True)
       + glob.glob(os.path.join(path, "**", "*.jpg"), recursive=True)
       + glob.glob(os.path.join(path, "**", "*.tif"), recursive=True))
print("图像文件数:", len(files))
img = Image.open(files[0]); print("尺寸/模式:", img.size, img.mode)

# torchvision Dataset 方式:
# from torchvision import datasets
# ds = datasets.ImageFolder(path)  # 要求 子目录=类别

目录组织与标注格式以源站说明和下载后实际文件为准。

数据说明

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a highly malignant inherited arrhythmogenic disorder. Type 1 CPVT (CPVT1) is caused by cardiac ryanodine receptor (RyR2) gene mutations resulting in abnormal calcium release from sarcoplasmic reticulum. Dantrolene, an inhibitor of sarcoplasmic Ca2+ release, has been shown to rescue this abnormal Ca2+ release in vitro. We assessed the antiarrhythmic efficacy of dantrolene in six patients carrying various RyR2 mutations causing CPVT. The patients underwent exercise stress test before and after dantrolene infusion. Dantrolene reduced the number of premature ventricular complexes (PVCs) on average by 74% (range 33-97) in four patients with N-terminal or central mutations in the cytosolic region of the RyR2 protein, while dantrolene had no effect in two patients with mutations in or near the transmembrane domain. Induced pluripotent stem cells (iPSCs) were generated from all the patients and differentiated into spontaneously beating cardiomyocytes (CMs). The antiarrhythmic effect of dantrolene was studied in CMs after adrenaline stimulation by Ca2+ imaging. In iPSC derived CMs with RyR2 mutations in the N-terminal or central region, dantrolene suppressed the Ca2+ cycling abnormalities in 80% (range 65-97) of cells while with mutations in or near the transmembrane domain only in 23 or 32% of cells. In conclusion, we demonstrate that dantrolene given intravenously shows antiarrhythmic effects in a portion of CPVT1 patients and that iPSC derived CM models replicate these individual drug responses. These findings illustrate the potential of iPSC models to individualize drug therapy of inherited diseases.
Trial Registration
EudraCT Clinical Trial Registry 2012-005292-14

许可

CC BY 4.0

引用

学术使用请引用 DOI 10.1371/journal.pone.0125366（source: https://plos.figshare.com/articles/dataset/Antiarrhythmic_Effects_of_Dantrolene_in_Patients_with_Catecholaminergic_Polymorphic_Ventricular_Tachycardia_and_Replication_of_the_Responses_Using_iPSC_Models/2666056）。