Comprehensive Phase 1 monotherapy data from the phase 1/2 TRESR trial will be presented at AACR on Monday, April 11, 2022 at 3:05 p.m. CT

Company to host conference call on Monday, April 11, 2022 at 6:30 p.m. ET

Repare Therapeutics Inc. (“Repare” or the “Company”) (Nasdaq: RPTX), a leading clinical-stage precision oncology company, today announced it has been selected for an oral presentation of clinical data from its ongoing Phase 1/2 TRESR (Treatment Enabled by SNIPRx) trial of RP-3500, a potent and selective oral small molecule inhibitor of ATR (Ataxia-Telangiectasia and Rad3-related protein kinase), as well as two additional poster presentations at the upcoming 2022 AACR Annual Meeting held in New Orleans on April 8-13, 2022.

The Company also announced that it will host a conference call with accompanying slides for analysts and investors on Monday, April 11, 2022 at 6:30 p.m. Eastern Time. The call and presentation will include RP-3500 data presented at the 2022 AACR Annual Meeting as well as additional data, representing an update from data in the Company’s abstract (referenced below), which reflects an earlier cut-off date.

“As with any new therapeutic class, differentiation among early product candidates is what ultimately determines success in the clinic and a path toward registration,” commented Lloyd M. Segal, President and Chief Executive Officer of Repare. “In the context of data published as recently as today, we are confident that RP-3500 is a highly differentiated and potentially leading ATR inhibitor. We continue to draw on a wealth of data from the comprehensive Phase 1 monotherapy module of our Phase 1/2 TRESR trial of RP-3500 to begin establishing its therapeutic potential. We look forward to seeing these data presented at AACR and discussing RP-3500’s ongoing and future development plans.”

Oral Presentation Details on RP-3500 Phase 1/2 TRESR Trial Results:

Title: Genomic and pathologic determinants of response to RP-3500, an ataxia telangiectasia and Rad3-related inhibitor (ATRi), in patients (pts) with DNA damage repair (DDR) loss-of-function (LOF) mutant tumors in the Phase 1/2 TRESR trial

Presenter: Dr. Timothy Yap, MBBS, Ph.D., FRCP, Medical Director, Institute for Applied Cancer Science, Associate Professor, Department of Investigational Cancer Therapeutics, Division of Cancer Medicine, MD Anderson Cancer Center, Houston, Texas

Abstract Number: CT030

Session Title: Clinical Trials Minisymposium - Patient Selection Strategies for Molecularly Targeted Agents in Clinical Trials

Date/ Time: Monday, Apr 11, 2022 at 3:05 PM – 3:15 PM CT

Poster Presentation Details on SNiPDx Panel for Synthetic Lethal Drug Discovery:

Title: Detection of biallelic loss of DNA repair genes in formalin-fixed, paraffin embedded (FFPE) tumor samples using a novel tumor-only sequencing panel with error correction

Presenter: Dominik Glodzik, Ph.D., Repare Therapeutics, Instructor in Biomedical Informatics, Harvard Medical School

Abstract Number: 2801

Session Title: Diagnostic Biomarkers

Date/ Time: Tuesday, April 12, 2022 at 9:00 AM CT

Poster Details on Preclinical Data for PKMYT1 Inhibitor RP-6306:

Title: RP-6306, a novel PKMYT1 inhibitor, demonstrates synthetic lethality as monotherapy and in combination with gemcitabine in CCNE1 amplified cancer cells

Presenter: Jimmy Fourtounis, Repare Therapeutics

Abstract Number: 5650

Session Title: Cell Cycle

Date/ Time: Friday, April 8, 2022 at 12:00 – 1:00 PM CT

Company Conference Call:

The Company will host a conference call with accompanying slides for analysts and investors on Monday, April 11, 2022 at 6:30 p.m. Eastern Time to further discuss the RP-3500 data presented at the 2022 AACR Annual Meeting. Repare’s executive management team will be joined by Timothy Yap, MBBS, PhD, FRCP, Principal Investigator and Medical Director, Institute for Applied Cancer Science, Associate Professor, Department of Investigational Cancer Therapeutics, Division of Cancer Medicine, MD Anderson Cancer Center, Houston, TX.

To access the call, please dial (877) 870-4263 (U.S. and Canada) or (412) 317-0790 (international) at least 10 minutes prior to the start time and ask to be joined to the Repare Therapeutics call. A live video webcast will be available in the Investor section of the Company’s website at https://ir.reparerx.com/news-and-events/events. A webcast replay will also be archived for at least 30 days.

About Repare Therapeutics’ SNIPRx® Platform

Repare’s SNIPRx® platform is a genome-wide CRISPR-based screening approach that utilizes proprietary isogenic cell lines to identify novel and known synthetic lethal gene pairs and the corresponding patients who are most likely to benefit from the Company’s therapies based on the genetic profile of their tumors. Repare’s platform enables the development of precision therapeutics in patients whose tumors contain one or more genomic alterations identified by SNIPRx® screening, in order to selectively target those tumors in patients most likely to achieve clinical benefit from resulting product candidates.

About Repare Therapeutics, Inc.

Repare Therapeutics is a leading clinical-stage precision oncology company enabled by its proprietary synthetic lethality approach to the discovery and development of novel therapeutics. The Company utilizes its genome-wide, CRISPR-enabled SNIPRx® platform to systematically discover and develop highly targeted cancer therapies focused on genomic instability, including DNA damage repair. The Company’s pipeline includes its lead product candidate RP-3500, a potential leading ATR inhibitor currently in Phase 1/2 clinical development, its second clinical candidate, RP-6306, a PKMYT1 inhibitor currently in Phase 1 clinical development, a Polθ inhibitor program, as well as eight other early-stage, pre-clinical programs. For more information, please visit reparerx.com.

SNIPRx® is a registered trademark of Repare Therapeutics Inc.

Forward-Looking Statements

This press release contains “forward-looking statements” within the meaning of the Private Securities Litigation Reform Act of 1995. All statements in this press release other than statements of historical facts are “forward-looking statements. These statements may be identified by words such as “aims,” “anticipates,” “believes,” “could,” “estimates,” “expects,” “forecasts,” “goal,” “intends,” “may,” “plans,” “possible,” “potential,” “seeks,” “will” and variations of these words or similar expressions that are intended to identify forward-looking statements, although not all forward-looking statements contain these words. Forward-looking statements in this press release include, but are not limited to, statements regarding the clinical development of the Company’s pipeline and its research and development programs, including the anticipated timing, anticipated patient enrollment, trial outcomes or associated costs of its Phase 1/2 TRESR clinical trial of RP-3500. These forward-looking statements are based on the Company’s expectations and assumptions as of the date of this press release. Each of these forward-looking statements involves risks and uncertainties that could cause the Company’s clinical development programs, future results or performance to differ materially from those expressed or implied by the forward-looking statements. Many factors may cause differences between current expectations and actual results, including the impacts of the COVID-19 pandemic on the Company’s business, clinical trials and financial position, unexpected safety or efficacy data observed during preclinical studies or clinical trials, clinical trial site activation or enrollment rates that are lower than expected, changes in expected or existing competition, changes in the regulatory environment, the uncertainties and timing of the regulatory approval process, and unexpected litigation or other disputes. Other factors that may cause the Company’s actual results to differ from those expressed or implied in the forward-looking statements in this press release are identified in the section titled "Risk Factors" in the Company’s Annual Report on Form 10-K for the year ended December 31, 2021 filed with the Securities and Exchange Commission (“SEC”) and the Québec Autorité des Marchés Financiers ("AMF") on March 1, 2022, and its other documents subsequently filed with or furnished to the SEC and AMF. The Company expressly disclaims any obligation to update any forward-looking statements contained herein, whether as a result of any new information, future events, changed circumstances or otherwise, except as otherwise required by law.

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