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ZielBio Receives Orphan Drug Designation for ZB131 for the Treatment of Cholangiocarcinoma

CHARLOTTESVILLE, Va.,/PRNewswire/ — ZielBio, Inc., a clinical-stage biotechnology company discovering new treatments for cancer and other serious diseases through its innovative drug discovery platform, today announced that the U.S. Food and Drug Administration (FDA) has granted Orphan Drug Designation to its lead program, ZB131, for the treatment of cholangiocarcinoma, a rare solid-tumor cancer originating from the bile duct system. ZB131 is a monoclonal antibody with a high affinity and specificity for cancer-specific plectin (CSP), a cell surface protein identified in a wide range of cancers that correlates with poor prognosis and aggressive tumors.

“Patients suffering from cholangiocarcinoma have few promising therapeutic options,” said Kimberly Kelly, Ph.D., founder and CEO of ZielBio. “Receiving this Orphan Drug Designation is a regulatory milestone for ZielBio that validates our sustained efforts to bring new treatments to underserved patients.”

ZielBio developed ZB131 to target CSP after discovering that, unlike in healthy cells where plectin exists in the cytoplasm, plectin presents on the surface of cancer cells, where it functions as a driver of tumor growth and metastasis. In preclinical studies, ZB131 demonstrated high specific binding to CSP and strong anti-cancer activity. A Phase 1/2 trial is underway to study the safety, tolerability, and efficacy of ZB131 in patients with solid tumors, including cholangiocarcinoma, pancreatic, and ovarian cancers. Additional study details are available at clinicaltrials.gov with the trial identifier NCT05074472.

FDA Orphan Drug Designation may be granted to investigational drugs or biological products which show promise in treating rare medical diseases or conditions that affect fewer than 200,000 people in the United States. About 8,000 people in the United States are diagnosed each year with cholangiocarcinoma, including both intrahepatic (inside the liver) and extrahepatic (outside the liver) bile duct cancers.

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