Forward-Looking Statements This presentation contains "forward-looking" statements that are based on our management's beliefs and assumptions and on information currently available to management. Forward-looking statemen

Forward-Looking Statements This

presentation contains "forward-looking" statements that are based on our management's beliefs and assumptions and on information currently available to management. Forward-looking statements include all statements other than statements of

historical fact contained in this presentation, including information concerning our business strategy, objectives and opportunities. Forward-looking statements are subject to known and unknown risks, uncertainties, assumptions and other factors

that may cause our actual results, performance or achievements to differ materially and adversely from those anticipated or implied by our forward-looking statements, including, but not limited to, those related to our dependence on third-party

clinical research organizations, manufacturers, suppliers and distributors; our ability to obtain necessary additional capital; our ability to obtain necessary regulatory approvals for our products and, if and when approved, market acceptance of our

products; the commercialization plans and expectations for commercializing mavodelpar (REN001) in the United States and rest of world, estimates of the number of patients impacted by PMM or LC-FAOD and who are appropriate for treatment with

mavodelpar, the potential benefits of mavodelpar, the financial impact or revenues from any commercialization we undertake, the impact of competitive products and therapies; our ability to attract and retain key employees; the costs of our

commercialization plans and development programs; the design, implementation and outcomes of our clinical trials; our ability to manage the growth and complexity of our organization; our ability to maintain, protect and enhance our intellectual

property; and our ability to continue to stay in compliance with applicable laws and regulations. You should refer to the section entitled "Risk Factors" set forth in our Annual Report on Form 10-K, Quarterly Reports on Form 10-Q and

other filings we make with the Securities and Exchange Commission (SEC) from time to time for a discussion of important factors that may cause our actual results to differ materially from those expressed or implied by our forward-looking statements.

You should not rely upon forward-looking statements as predictions of future events. Neither we nor any other person assumes responsibility for the accuracy and completeness of the forward-looking statements. We undertake no obligation to update any

forward-looking statements after the date of this presentation except as may be required by law. This presentation also contains estimates and other statistical data made by independent parties and by us relating to market size and growth and other

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operate are necessarily subject to a high degree of uncertainty and risk. The trademarks included herein are the property of the owners thereof and are used for reference purposes only. Mavodelpar is an investigational drug product candidate that is

under clinical investigation, and which has not yet been approved for marketing by the U.S. Food and Drug Administration, European Medicines Agency, or any other global regulatory agency. No representation is made as to the safety or effectiveness

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Investment Highlights Financial

Overview Mitochondrial Diseases R&D Milestones Mavodelpar (REN001) Preliminary evidence of efficacy and tolerability from four clinical trials Favorable guidance from U.S. and European regulatory agencies Rare diseases with high unmet medical

need Myopathy and reduced life expectancy common Pivotal trial in primary mitochondrial myopathy (PMM) with data 4Q23 Expansion into two additional mitochondrial populations in 2023 $116 million cash, cash equivalents, and short-term investments as

of Sept 30, 2022 Fundamental investor base Rx

Mavodelpar Overview Increases

transcription of genes central to mitochondrial function Drives production of new mitochondria Increases oxidation of fatty acids and cellular energy production PPAR EC50 = 31nM PPARa EC50 > 10 M PPAR EC50 > 10 M Biology

Potent and selective agonist of peroxisome proliferator-activated receptor delta (PPAR ) Regulates generation of cellular energy Present in multiple tissue types including muscle, brain, kidney, and liver Activation in response to increased

cellular energy needs

Nutrient Utilization by Mitochondria %

of Total Energy 100 0 Phosphocreatine Carbohydrates Fatty acids 2 4 6 10 8 30 12 0 Minutes Fatty acid oxidation becomes the predominant nutrient source for generation of cellular energy by the mitochondria during periods of increased energy demand

Patients with PMM and LC-FAOD experience myopathy, weakness, fatigue, or deterioration in muscle due to impaired mitochondrial energy production

Reneo Pharmaceuticals Pipeline Reneo is

initially developing mavodelpar for patients with rare genetic mitochondrial diseases that typically present with myopathy and have a high unmet medical need Preclinical Phase 1 Phase 2/3 Approved 2023 Anticipated Milestones PPM primary

mitochondrial myopathies Complete enrollment of pivotal trial in mtDNA PMM (1Q23) nDNA PMM program update "STRIDE AHEAD" (1Q23) Topline data from pivotal trial in mtDNA PMM (4Q23) LC-FAOD long-chain fatty acid oxidation disorders FDA

guidance on LC-FAOD program (1Q23) mitochondrial DNA (mtDNA) mutations/deletions nuclear DNA (nDNA) mutations/deletions nuclear DNA (nDNA) mutations/deletions

Reneo has operations currently in US

and UK Prevalence in Key Rare Disease Markets Patients in Europe (Northern/Western) United Nations World Population Prospects, Rev.166200 (2019) Gorman G. et al., Ann Neurol. 77(5): 753-759 (2015) Merritt J.L. et al., Ann Transl Med. 6(24):

473 (2018) Patients in China Patients in Japan Patients in US mtDNA PMM: 66,200 LC-FAOD: 5,000 Patients in Brazil mtDNA PMM: 82,500 LC-FAOD: 6,200 mtDNA PMM: 42,500 LC-FAOD: 3,200 mtDNA PMM: 23,500 LC-FAOD: 1,900 mtDNA PMM: 287,900 LC-FAOD:

Leg Immobilization Study: Overview

Primary Objective Evaluate safety and tolerability of 28 days of mavodelpar in healthy volunteers Secondary Objectives Changes in muscle strength and size Changes in expression of PPAR regulated genes involved in mitochondrial function and

biogenesis 29 1 14 Day Mavodelpar 100 mg BID (n=12) Baseline 49 Placebo (n=12) Follow-up Leg immobilized for 14 days Randomized, blinded, placebo-controlled clinical trial in adult subjects during and after leg immobilization Davies M. et al., J

Clin Trials. 12:495 (2022)

Leg Immobilization Study: Changes in

Muscle Strength No serious adverse events (SAEs) related to mavodelpar treatment Treatment-emergent adverse events (TEAEs) were similar between subjects treated with mavodelpar or placebo Subjects treated with mavodelpar had substantially more leg

strength compared placebo subjects Greater preservation of muscle strength following immobilization Greater increase in muscle strength one week after immobilization Davies M. et al., J Clin Trials. 12:495 (2022) (p-value from a mixed model with

baseline value as covariate)

Leg Immobilization Study: Changes in

Gene Expression Subjects treated with mavodelpar showed increases in expression of PPAR -regulated genes of interest Pyruvate dehydrogenase lipoamide kinase isozyme 4 (PDK4) encodes for a protein that plays a key role in regulation of glucose

and fatty acid metabolism Angiopoietin-like 4 (ANGPTL4) encodes for a protein that is directly involved in regulating lipid metabolism Solute carrier family 25 member 34 (SLC25A34) encodes for a protein that is known to transport molecules across

the mitochondrial membrane Davies M. et al., J Clin Trials. 12:495 (2022)

Primary Mitochondrial Myopathies

(PMM) Characteristics PMMs are rare disorders caused by mutations within mitochondrial DNA (mtDNA) or nuclear DNA (nDNA) Mutations hamper the ability of mitochondria to generate energy Most pronounced in tissues with high energy demand (muscle,

brain, and heart) Symptoms Debilitating fatigue Myopathy Exercise intolerance Muscle pain Severe lack of endurance Reduced life expectancy Adult Prevalence* 9.6:100,000 (mtDNA) 2.9:100,000 (nDNA) Current Treatments No approved therapies

Over-the-counter vitamins and supplements commonly used Gorman G. et al., Ann Neurol. 77(5): 753-759 (2015)

PMM Phase 1b Study: Overview

Open-label clinical trial in adult PMM subjects with mtDNA defects and myopathy Primary Objective Evaluate safety and tolerability of 12 weeks of treatment with mavodelpar in PMM patients Secondary Objectives Evaluate safety and tolerability of 48

weeks of mavodelpar Explore changes in clinical outcome such as exercise tests, oxygen consumption, and symptoms Baseline 4 12 1 2 8 Week 36-week extension (optional) Mavodelpar 100 mg QD (n=23) Mavodelpar 100 mg QD

Mean increase over baseline in 12MWT

distance of 104.4 meters, 95% CI [53.1, 155.6] 13 patients (77%) had greater than a 50-meter increase 12MWT distance PMM Phase 1b Study: Changes in 12-Minute Walk Test (12MWT)

PMM Phase 1b Study: Changes in

Walking Velocity Walking velocity of PMM patients decreased at each 3-minute period during the 12MWT Following 12-weeks of treatment with mavodelpar, walking velocity increased compared to baseline The greatest increase in walking velocity occurred

during the second half of the 12MWT +25% +22%

PMM Phase 1b Study: Changes in Peak

Oxygen Consumption (VO2) Mean increase over baseline in peak VO2 of 1.7 mL/min/kg, 95% CI [-0.3, 3.7] 10 patients (59%) had an increase in peak VO2 of 0.9 mL/min/kg or greater

PMM Phase 1b Study: Changes in

Symptoms Mean change in SF-36 energy/fatigue score of 11.2, 95% CI [1.2, 21.2] Mean change in Modified Fatigue Impact Scale of -10.5, 95% CI [-16.3, -4.6] Mean change in Brief Pain Inventory of -1.0, 95% CI [-0.2, -1.9]

PMM Phase 1b Study: Changes in Gene

Transcription Muscle biopsies were performed at baseline and after 12 weeks of treatment with mavodelpar Differential gene expression was performed on biopsies from seven (7) subjects that had sufficient sample quantity and quality for analysis at

baseline and week 12 A statistically significant increase over baseline was observed in the expression of PDK4, ANGPTL4, and SLC25A34 Gene expression data is consistent with the findings from the leg immobilization study Expression of

PPAR -regulated genes from muscle biopsies (PMM patients)

Primary Objective Change from

baseline to week 24 in distance walked during 12MWT Secondary Objectives Changes from baseline to week 24 in MFIS physical sub-scale and PGIC scores 30 second sit-to-stand test, step count, PGIS score, SF-36, MFIS total, cognitive and psychosocial

sub-scale, BPI severity and pain interference, and WPAI:SHP January 2023 Status STRIDE enrollment over 90%; majority of ex-US participants (84%) entered STRIDE AHEAD Randomized, double-blind, placebo-controlled clinical trial in adult subjects with

mtDNA defects and myopathy PMM Phase 2b Study: Overview 24 2 18 Week Mavodelpar 100 mg QD (n=100) Placebo (n=100) Mavodelpar 100 mg QD 4 12 2-year open-label extension (ex-US) Screening Baseline "STRIDE" "STRIDE

Long-Chain Fatty Acid Oxidation

Disorders (LC-FAOD) Characteristics LC-FAOD are rare disorders caused by mutations within nuclear DNA (nDNA) Inherited genetic errors of metabolism resulting in the inability to use dietary long-chain fatty acids as energy sources Mutations in genes

that code for enzymes that perform long-chain fatty acid oxidation Symptoms Young children- lethargy, liver dysfunction, hypoglycemia, high risk for sudden death, cardiomyopathy Older patients - limited endurance, muscle aches,

rhabdomyolysis Prevalence* 1:120,000 to 1:42,500 (VLCAD deficiency) 1:150,000 to 1:110,000 (LCHAD deficiency) Current Treatments DOLJOVI , a fatty acid supplement similar to medium chain triglyceride oil (MCT) Therapy includes a fat-restricted

diet, supplementing with short or medium chain fatty acids Merritt J.L. 2nd et al., Ann Transl Med 6(24):473 (2018)

LC-FAOD Phase 1b Study: Overview

Primary Objective Evaluate safety and tolerability of 12 weeks of treatment with mavodelpar in LC-FAOD patients Secondary Objectives Explore changes in clinical outcome such as exercise tests and symptoms after 12 weeks of treatment with mavodelpar

Open-label clinical trial in adult LC-FAOD subjects with mutations in LCHAD, CPTs, VLCAD, and TFP genes 4 12 1 2 8 Week Mavodelpar 50 mg QD (n=3) or 100 mg QD (n-21) Baseline

LC-FAOD Phase 1b Study: 12MWT, SF-36

Energy/Fatigue, and MFIS Mean increase over baseline in 12MWT distance over 50 meters in LCHAD and CPT2 genotypes LC-FAOD program to move forward; FDA feedback on Phase 2 trial design pending Preclinical 12MWT [meters] SF-36 Energy/Fatigue MFIS

Total Genotype n Baseline Change n Baseline Change n Baseline Change LCHAD 5 547.7 (133.4) 73.7 (18.0) 5 44.3 (10.4) 19.5 (11.7) 5 32.8 (6.5) -9.8 (4.2) CPT2 6 949.6 (119.1) 51.9 (49.4) 6 57.7 (3.2) 0.8 (4.9) 6 23.5 (6.7) 1.0 3.3) VLCAD 5 864.3

(65.1) -36.7 (42.1) 5 57.3 (9.3) -17.8 (7.8) 5 17.8 (6.8) 15.6 (8.5)

Mavodelpar Roadmap 2H 2023 LC-FAOD

Phase 2 program update mtDNA PMM Phase2b STRIDE enrollment complete 1H 2023 1H 2024 mtDNA PMM OLE safety STRIDE AHEAD interim results mtDNA PMM Phase2b STRIDE topline data mtDNA PMM Program NDA/MAA filings nDNA PMM Study program update

To learn more, please contact:

Danielle Spangler Investor Relations dspangler@reneopharma.com