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The role of BiP and the IRE1α-XBP1 axis in rhabdomyosarcoma pathology

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DataONE2025-03-05 更新2025-04-26 收录
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Background: Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children, and is associated with a poor prognosis in patients presenting with recurrent or metastatic disease. The unfolded protein response (UPR) plays pivotal roles in tumor development and resistance to therapy, including RMS. Methods: In this study, we used immunohistochemistry and a tissue microarray (TMA) on human RMS and normal skeletal muscle to evaluate the expression of key UPR proteins (GRP78/BiP, IRE1α and cytosolic/nuclear XBP1 (spliced XBP1-sXBP1)) in the four main RMS subtypes: alveolar (ARMS), embryonal (ERMS), pleomorphic (PRMS) and sclerosing/spindle cell (SRMS) RMS. We also investigated the correlation of these proteins with the risk of RMS and several clinicopathological indices, such as lymph node involvement, distant metastasis, tumor stage and tumor scores. Results: Our results revealed that the expression of BiP, sXBP1, and IRE1α, but not cytosolic XBP1, are significantly associated with..., 2.1. Tissue Microarray (TMA) The tissue microarray (TMA) analysis was conducted on rhabdomyosarcoma (RMS) and normal (control) striated muscle samples. A rhabdomyosarcoma tissue array kit containing 104 cases and 208 cores was utilized, purchased from US Biomax, Inc. (Catalog no. SO2082a; RKV, MD, USA). This array provides detailed information on tumor characteristics, including TNM (tumor, node, metastasis) staging, clinical stages, and pathology grades, ensuring comprehensive clinical and pathological data for each sample. The TMA includes cores from four subtypes of RMS: 15 cases of spindle cell/sclerosing RMS (SRMS) 27 cases of embryonal RMS (ERMS) 30 cases of pleomorphic RMS (PRMS) 24 cases of alveolar RMS (ARMS) Additionally, it includes 8 normal skeletal muscle tissue cores as controls, with all samples processed in duplicate. Each of the 208 tissue cores is embedded on a single slide, with two cores derived from each case or control. Detailed histopathological characteri..., , # The Role of BiP and the IRE1α-XBP1 Axis in Rhabdomyosarcoma Pathology [https://doi.org/10.5061/dryad.d2547d8c6](https://doi.org/10.5061/dryad.d2547d8c6) It is Tissue microarray data ## Code/software Any software for image (adobe photoshop, image j) and excel/csv.  ## Description of the data and file structure **Detailed Description of the BIP File:** ### **1. File Name: TMA Patients Data - Cancers Paper - BIP.csv** This dataset presents patient-level data related to tissue microarrays (TMA) used to study the **cytosolic expression of BIP (Binding Immunoglobulin Protein)**, an essential molecular chaperone in the endoplasmic reticulum involved in protein folding and stress responses. The cytosolic score corresponds to the **quantified expression of BIP in the cytosol**, which is important in understanding cancer development and progression, particularly in rhabdomyosarcoma and other cancers. --- **2. Structure of the Dataset** * **Columns Overview:** * Position: Position i...
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2025-03-06
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