CCN1

NORTH CHICAGO, Ill., May 1, 2025 /PRNewswire/ -- BLR Bio, a biotechnology company in Rosalind Franklin University's Helix 51 incubator, announced new data on its novel therapy for systemic sclerosis and lung fibrosis at the British Society for Rheumatology (BSR) Annual Conference this week in Manchester, UK. BLR Bio CEO and CSO Bruce Riser, a renowned expert in fibrotic diseases, pictured in his lab at Rosalind Franklin University's Helix 51 biomedical incubator. Interstitial Lung Disease (ILD) is a devastating disorder, encompassing a group of chronic lung conditions that affect the tissue between the air sacs in the lungs. ILD conditions can include Idiopathic pulmonary fibrosis (IPF), which can result from systemic sclerosis. The estimated prevalence in the United States is 654,852, according to a February 2024 publication in the journal CHEST. "These patients are in urgent need of a therapy that can halt or reverse this disease without the threat of serious side effects," said BLR Bio CEO and CSO Dr. Bruce Riser. In the new study, presented by Dr. Andrew Leask at the University of Saskatchewan in Canada, BLR 200, the company's lead compound, demonstrated the ability to reduce key disease tissue markers for fibrotic lung disease. Reductions were shown in Ashcroft scores, collagen levels, and both fibronectin-1 (FN-1) and profibrotic CCN4, signaling molecules involved in the development of scar tissue. Dr. Leask is a leading expert in fibrosis and CCN proteins and a long-term collaborator with BLR Bio. "Our data shows the reductions in Ashcroft scores and collagen levels appear to be more pronounced than those seen with other therapies on the market and in development," Dr. Riser said. "Combined with prior observations modeling scleroderma skin fibrosis, BLR-200 has shown the potential to address both skin and lung fibrosis. It appears to be the only drug in development that reduces expression of all three CCN proteins — CCN1, CCN2, CCN4 — that play a crucial role in the activation of fibroblast cells critical for initiating and maintaining fibrosis." Dr. Joseph DiMario, RFU Interim VP for Research, noted that BLR Bio was one of the first tenants of Helix 51. "We are very pleased to see the progress the company is making toward the development of life-changing medicines," he said. About Rosalind Franklin University Rosalind Franklin University of Medicine and Science embodies the spirit of inquiry and excellence modeled by its namesake Dr. Rosalind Franklin, whose Photo 51 was crucial to solving the structure of DNA. Recognized for its research in areas including neuroscience, brain-related diseases, inherited disorders, diabetes, obesity, and gait and balance, RFU encompasses the Chicago Medical School, College of Health Professions, College of Nursing, College of Pharmacy, School of Graduate and Postdoctoral Studies and the Dr. William M. Scholl College of Podiatric Medicine. Learn more at rosalindfranklin.edu. About BLR Bio Learn more at blrbio.com. Office of Marketing and Communications [email protected] SOURCE Rosalind Franklin University of Medicine and Science WANT YOUR COMPANY'S NEWS FEATURED ON PRNEWSWIRE.COM? 440k+ Newsrooms & Influencers 9k+ Digital Media Outlets 270k+ Journalists Opted In GET STARTED

A loss of salt and body fluid can stimulate kidney regeneration and repair in mice, according to a new study. This innate regenerative response relies on a small population of kidney cells in a region known as the macula densa (MD), which senses salt and exerts control over filtration, hormone secretion, and other key functions of this vital organ. A loss of salt and body fluid can stimulate kidney regeneration and repair in mice, according to a USC Stem Cell study. This innate regenerative response relies on a small population of kidney cells in a region known as the macula densa (MD), which senses salt and exerts control over filtration, hormone secretion, and other key functions of this vital organ. The scientists focused on how healthy kidneys originally evolved from the primitive kidney structure of the fish into more complicated and more efficiently working kidneys to absorb more salt and water. The research team fed lab mice a very low salt diet, along with a commonly prescribed drug called an ACE inhibitor that furthered lowered salt and fluid levels. In the region of the MD, the scientists observed regenerative activity, which they could block by administering drugs that interfered with signals sent by the MD. This underscored the MD's key role in orchestrating regeneration. In the mouse MD cells, the scientists also identified specific signals from certain genes, including Wnt, NGFR, and CCN1, which could be enhanced by a low-salt diet to regenerate kidney structure and function. In keeping with these findings in mice, the activity of CCN1 was found to be greatly reduced in patients with chronic kidney disease (CKD). To test the therapeutic potential of these discoveries, the scientists administered CCN1 to mice with a type of CKD known as focal segmental glomerulosclerosis. They also treated these mice with MD cells grown in low-salt conditions. Both approaches were successful, with the MD cell treatment producing the biggest improvements in kidney structure and function. "Our personal and professional mission is to find a cure for kidney disease, a growing global epidemic affecting one out of seven adults, which translates to 850 million people worldwide or about 2 million in the Los Angeles area," said Peti-Peterdi, a professor of physiology, neuroscience and medicine at the Keck School of Medicine of USC. "Currently, there is no cure for this silent disease. By the time kidney disease is diagnosed, the kidneys are irreversibly damaged and ultimately need replacement therapies, such as dialysis or transplantation." To address this growing epidemic, Peti-Peterdi, first author Georgina Gyarmati, and their colleagues took a highly non-traditional approach. As opposed to studying how diseased kidneys fail to regenerate, the scientists focused on how healthy kidneys originally evolved. "From an evolutionary biology perspective, the primitive kidney structure of the fish turned into more complicated and more efficiently working kidneys to absorb more salt and water," said Peti-Peterdi, who also directs the Multi-Photon Microscopy Core at the Zilkha Neurogenetic Institute (ZNI). "This was necessary for adaptation to the dry land environment when the animal species moved from the salt-rich seawater. And that's why birds and mammals have developed MD cells and this beautiful, bigger, and more efficient kidney structure to maintain themselves and functionally adapt to survive. These are the mechanisms that we are targeting and trying to mimic in our research approach." With this evolutionary history in mind, the research team fed lab mice a very low salt diet, along with a commonly prescribed drug called an ACE inhibitor that furthered lowered salt and fluid levels. The mice followed this regimen for up to two weeks, since extremely low salt diets can trigger serious health problems if continued long term. In the region of the MD, the scientists observed regenerative activity, which they could block by administering drugs that interfered with signals sent by the MD. This underscored the MD's key role in orchestrating regeneration. When the scientists furthered analyzed mouse MD cells, they identified both genetic and structural characteristics that were surprisingly similar to nerve cells. This is an interesting finding, because nerve cells play a key role in regulating the regeneration of other organs such as the skin. In the mouse MD cells, the scientists also identified specific signals from certain genes, including Wnt, NGFR, and CCN1, which could be enhanced by a low-salt diet to regenerate kidney structure and function. In keeping with these findings in mice, the activity of CCN1 was found to be greatly reduced in patients with chronic kidney disease (CKD). To test the therapeutic potential of these discoveries, the scientists administered CCN1 to mice with a type of CKD known as focal segmental glomerulosclerosis. They also treated these mice with MD cells grown in low-salt conditions. Both approaches were successful, with the MD cell treatment producing the biggest improvements in kidney structure and function. This might be due to the MD cells secreting not only CCN1, but also additional unknown factors that promote kidney regeneration. "We feel very strongly about the importance of this new way of thinking about kidney repair and regeneration," said Peti-Peterdi. "And we are fully convinced that this will hopefully end up soon in a very powerful and new therapeutic approach." Additional co-authors are Urvi Nikhil Shroff, Anne Riquier-Brison, Dorinne Desposito, Audrey Izuhara, Sachin Deepak, Alejandra Becerra Calderon, James L. Burford, Hiroyuki Kadoya, Ju-Young Moon, Yibu Chen, Nariman Ahmadi, Berislav V. Zlokovic, and Inderbir S. Gill from USC; Wenjun Ju and Matthias Kretzler from the University of Michigan; Sean D. Stocker from the University of Pittsburgh School of Medicine; Markus M. Rinschen from the University of Cologne; Lester Lau from the University of Illinois at Chicago; Daniel Biemesderfer from Yale University School of Medicine; Aaron W. James from Johns Hopkins University; and Liliana Minichiello from the University of Oxford. This work was federally funded by the National Institutes of Health (grants DK064324, DK123564, DK135290, S10OD021833, and 2P30-DK-081943) and further supported by an American Heart Association predoctoral research fellowship (grant 19PRE34380886). Disclosure: Peti-Peterdi and Gyarmati are cofounders of Macula Densa Cell LLC, a biotechnology company that develops therapeutics to target MD cells for a regenerative treatment for CKD. Macula Densa Cell LLC has a patent entitled "Targeting macula densa cells as a new therapeutic approach for kidney disease" (US patents 10,828,374 and 11,318,209). Gill declares equity interest in OneLine Health and Karkinos.

CTCS HARVESTED BY PARSORTIX UNCOVER NOVEL METASTATIC BIOMARKER Parsortix system able to harvest difficult-to-detect mesenchymal CTCs Metastatic biomarker Cyr61 found by researchers in 40% of breast cancer CTCs may be a novel therapeutic target in treating drug-resistant breast cancers GUILDFORD, SURREY / ACCESSWIRE / February 18, 2021 / ANGLE plc (AIM:AGL OTCQX:ANPCY), a world-leading liquid biopsy company, is pleased to announce that the University Medical Centre Hamburg-Eppendorf, Germany, has published new research in the peer reviewed journal Cancers, that demonstrates the ability of the Parsortix® system to harvest circulating tumour cells (CTCs) with a mesenchymal phenotype, which can be used to detect the metastatic biomarker cysteine-rich angiogenetic inducer 61 (Cyr61) in breast cancer patients. The Cyr61 protein is a potent regulator of many cellular functions important in cancer development, including cell proliferation, survival/apoptosis, senescence, motility/chemotaxis, invasion into the extracellular matrix, gene expression, differentiation, and angiogenesis. Cyr61 is expressed in most forms of solid tumours and in breast cancer, potentially playing a role in tumour progression, metastatic expansion, and the development of resistance to anti-cancer drugs including endocrine therapies. In this study, researchers found that Cyr61 was over-expressed in 43% of CTCs isolated from 35 breast cancer patients. In addition, they were able to demonstrate in breast cancer cell-lines that the loss of Cyr61 reduced the viability of tumour cells by decreasing cancer cell proliferation and cell survival. As such, Cyr61 might serve as a novel marker for CTCs and disseminated tumour cells (DTCs) similar to those found in the bone marrow and help identify cells with a more aggressive phenotype. Furthermore, Cyr61 could represent a target for the development of novel anti-metastatic drugs. In the US, breast cancer is the most frequently diagnosed cancer in women with an estimated 276,480 new cases recorded in 2020. Despite new treatment options and advances in patient management protocols, some 30% of women initially diagnosed with earlier stages of breast cancer eventually develop recurrent advanced or metastatic disease. Although the prognosis of patients with metastatic breast cancer (MBC) has significantly improved, metastatic disease remains largely incurable with median survival of three years. CTCs, as a liquid biopsy, have significant potential as a complementary prognostic and diagnostic tool for clinicians. Access to tumour cells from a peripheral blood sample provides non-invasive, real-time monitoring of cancer patients, giving insight into clonal evolution during tumour metastasis. Critically, the analysis of CTCs from a blood sample has the potential to provide actionable information for the approximately 50% of all MBC patients who are unable to have a solid tissue biopsy of the metastatic site. This study demonstrates how CTCs can provide insight into molecular mechanisms of cancer cell resistance and metastatic spread, which may have potential as a novel therapeutic target in treating drug-resistant breast cancers. This research has been published in the peer-reviewed publication Cancers and may be accessed via . ANGLE Founder and Chief Executive, Andrew Newland, commented: "This new study observed that mesenchymal DTCs are strongly positive for PD-L1 and Cyr61 and that Cyr61/PD-L1 double-positive DTCs may escape the immune system and establish distant metastasis. Cyr61 might therefore serve as a novel biomarker for a subset of CTCs and DTCs with high plasticity in breast cancer. In addition, Cyr61 might represent an interesting anti-metastatic target that could be explored in future studies. Use of the Parsortix system in uncovering potential new therapeutic targets through its ability to harvest mesenchymal as well as epithelial CTCs for analysis provides yet further evidence of its advantages over other liquid biopsy approaches. ANGLE's ability to provide this actionable information should prove highly attractive to drug developers looking for new approaches for hard-to-treat cancers and bodes well for the forthcoming launch of our new pharmaceutical services business." For further information on ANGLE: ANGLE plc +44 (0) 1483 343434 Andrew Newland, Chief Executive Ian Griffiths, Finance Director Andrew Holder, Head of Investor Relations finnCap Ltd (NOMAD and Joint Broker) Corporate Finance - Carl Holmes, Simon Hicks, Teddy Whiley ECM - Alice Lane, Sunila de Silva +44 (0)20 7220 0500 WG Partners (Joint Broker) Nigel Barnes, Nigel Birks, Andrew Craig, Chris Lee +44 (0) 203 705 9330 FTI Consulting Simon Conway, Ciara Martin Matthew Ventimiglia (US) +44 (0) 203 727 1000 +1 (212) 850 5624 For Frequently Used Terms, please see the Company's website on Notes for editors About ANGLE plc ANGLE is a world leading liquid biopsy company with sample-to-answer solutions. ANGLE's proven patent protected platforms include a circulating tumor cell (CTC) harvesting technology and a downstream analysis system for cost effective, highly multiplexed analysis of nucleic acids and proteins. ANGLE's cell separation technology is called the Parsortix® system, and it enables a liquid biopsy (a simple blood test) to be used to provide the cells of interest to the user in a format suitable for multiple types of downstream analyses. The system is based on a microfluidic device that captures cells based on a combination of their size and compressibility. The system is epitope independent and can capture all types of CTCs as well as CTC clusters in a viable form (alive). CTCs enable the complete picture of a cancer to be seen as being an intact cell they allow DNA, RNA and protein analysis and thus provide comparable analysis to a tissue biopsy. Because CTC analysis is a non-invasive process, unlike tissue biopsy, it can be repeated as often as needed. This is important because cancer develops and changes over time and there is a clear medical need for up-to-date information on the status. In addition, the live CTCs harvested can be cultured, which offers the potential for testing response to drugs outside the patient. The Parsortix technology is the subject of 26 granted patents in Europe, the United States, China, Australia, Canada, India, Japan and Mexico with three extensive families of patents are being progressed worldwide. The Parsortix system has a CE Mark in Europe for the indicated use and, in the United States, a De Novo Submission has been made to FDA for the Parsortix® PC1 system seeking FDA clearance with Class II Classification for use with metastatic breast cancer patients. FDA clearance is seen as the global standard. ANGLE is seeking to be the first ever FDA cleared system for harvesting CTCs for subsequent analysis. ANGLE has also completed two separate 200 subject clinical studies under a program designed to develop an ovarian cancer pelvic mass triage test, with the results showing best in class accuracy (AUC-ROC) of 95.1%. The pelvic mass triage assay has undergone further refinement and optimisation and is currently in the process of a 200 patient clinical verification study. ANGLE's technology for the multiplex evaluation of proteins and nucleic acids of all types is called the HyCEADTM Ziplex® platform and is based on a patented flow through array technology. It provides for low cost, highly multiplexed, rapid and sensitive capture of targets from a wide variety of sample types. A proprietary chemistry approach (the HyCEAD method) allows for the capture and amplification of over 100 biomarkers simultaneously in a single reaction. The HyCEAD Ziplex system is extremely sensitive and is ideal for measuring gene expression and other markers directly from Parsortix harvests and was used in the ovarian cancer pelvic mass triage test to achieve best in class accuracy (AUC-ROC) of 95.1%. ANGLE's proprietary technologies can be combined to provide automated, sample-to-answer results in both centralised laboratory and point-of-use cartridge formats. ANGLE has established formal collaborations with world-class cancer centres and major corporates such as Abbott, Philips and QIAGEN, and works closely with leading CTC translational research customers. These Key Opinion Leaders (KOLs) are working to identify applications with medical utility (clear benefit to patients), and to secure clinical data that demonstrates that utility in patient studies. The body of evidence as to the benefits of the Parsortix system is growing rapidly from our own clinical studies in metastatic breast cancer and ovarian cancer and also from KOLs with 41 peer-reviewed publications and numerous publicly available posters, available on our website. This information is provided by Reach, the non-regulatory press release distribution service of RNS, part of the London Stock Exchange. Terms and conditions relating to the use and distribution of this information may apply. For further information, please contact rns@lseg.com or visit . SOURCE: ANGLE plc View source version on accesswire.com: