Larimar Therapeutics Reports Second Quarter 2021 Operating and Financial Results - Reported positive proof-of-concept and dose response data from Phase 1 program evaluating CTI-1601 in patients with Friedreich s ataxia (

Larimar Therapeutics Reports Second Quarter 2021 Operating and Financial Results

- Reported positive proof-of-concept and dose response data

from Phase 1 program evaluating CTI-1601 in patients with Friedreich s ataxia (FA)

Subcutaneous injections of CTI-1601 at doses of 50 mg or 100 mg resulted in frataxin levels in buccal cells of FA patients that were at or in excess of those that would be expected in phenotypically normal

heterozygous carriers

- Phase 1 safety data indicate that repeated subcutaneous injections of

CTI-1601 were generally well tolerated at doses up to 100 mg administered daily for 13 days

- Completed dosing in July in 180-day non-human primate

toxicology study designed to support extended dosing of CTI-1601

- Cash and investments of

$70.6 million as of June 30, 2021

- Closed $20 million equity financing on July 2, 2021

Bala Cynwyd, PA, August 12, 2021 Larimar Therapeutics, Inc. ( Larimar ) (Nasdaq: LRMR), a clinical-stage biotechnology company focused

on developing treatments for complex rare diseases, today reported its second quarter and year to date June 30, 2021 operating and financial results.

We finished the second quarter in a strong financial position and with a compelling clinical data set that demonstrates

proof-of-concept for CTI-1601, which to our knowledge is the only clinical-stage candidate designed to address the root cause of

Friedreich s ataxia, said Carole Ben-Maimon, MD, President and Chief Executive Officer of Larimar. These positive Phase 1 data along with non-clinical pharmacology data demonstrate proof-of-concept, and CTI-1601 s differentiated mechanism of action helped us to earn a PRIME designation from the European

Medicines Agency, providing us with valuable regulatory benefits and important external validation. Looking forward, we continue to collect and analyze data from our 180-day

non-human primate toxicology study and remain confident that there is a path forward through the resolution of the CTI-1601 clinical hold and towards the initiation of

our Jive open-label extension and pediatric multiple ascending dose trials.

Second Quarter 2021 Highlights

Under an Equity Distribution

Agreement with an investment bank, the Company may sell up to an aggregate of $50,000,000 shares of common stock from time to time in connection with an at the market program. In July 2021, the Company sold an additional 2,342,720 shares

under the agreement for net proceeds of $19.9 million. The Company can raise an additional $29.2 million under this program.

Second Quarter 2021 Financial Results

June 30, 2021, the Company had cash and cash equivalents totaling $70.6 million.

The Company reported a net loss for the second quarter of 2021

of $12.6 million, or $0.79 per share, compared to a net loss of $11.3 million, or $1.21 per share, for the second quarter of 2020.

Research and development expenses for the second quarter of 2021 were $9.1 million compared to

$8.9 million for the second quarter of 2020. The increase in research and development expenses compared to the prior year period was primarily driven by higher non-clinical costs associated with assay

development and toxicology studies, an increase in clinical trial costs, an increase in personnel related costs due to headcount additions in our research and development functions, an increase in stock compensation expense associated with stock

option grants made in the second half of 2020 and thus far in 2021, partially offset by a decrease of clinical supply manufacturing costs.

administrative expenses for the second quarter of 2021 were $3.4 million, compared to $2.5 million for the second quarter of 2020. The increase in general and administrative expenses as compared to the prior year period was primarily

driven by an increase in personnel related costs due to increased headcount, an increase in stock-based compensation associated with stock option grants made in the second half of 2020 and thus far in 2021, an increase in professional fees primarily

associated with insurance costs, legal and consulting fees as a result of operating as a public company.

For the first half of 2021, the Company reported

a net loss of $24.7 million, or $1.54 per share, compared to a net loss of $18.1 million, or $2.33 per share for the same period in 2020.

Research and development expenses for the first half of 2021 were $18.1 million compared to $13.9 million for the first half of 2020. The increase

in research and development expenses compared to the prior year period was primarily driven by an increase in clinical trial costs, higher non-clinical costs associated with the assay and toxicology studies,

an increase in personnel related costs due to headcount additions in our research and development functions, an increase in stock compensation expense associated with stock option grants made in the second half of 2020 and thus far in 2021,

partially offset by a decrease of clinical supply manufacturing costs.

General and administrative expenses for the first half of 2021 were

$6.6 million, compared to $4.2 million for the first half of 2020. The increase in general and administrative expenses as compared to the prior year period was primarily driven by an increase in personnel related costs due to increased

headcount, an increase in stock-based compensation associated with stock option grants made in the second half of 2020 and thus far in 2021, an increase in professional fees primarily associated with insurance costs, recruiting expenses, legal and

consulting fees as a result of operating as a public company, partially offset by a decrease in accounting and audit costs related to additional years under audit in the first quarter 2020.

About Larimar Therapeutics

Larimar Therapeutics,

Inc. (Nasdaq: LRMR) is a clinical-stage biotechnology company focused on developing treatments for complex rare diseases. Larimar s lead compound, CTI-1601, is currently being evaluated in a Phase 1

clinical program in the U.S. as a potential treatment for Friedreich s ataxia. Larimar also plans to use its intracellular delivery platform to design other fusion proteins to target additional rare diseases characterized by

deficiencies in intracellular bioactive compounds. For more information, please visit: https://larimartx.com.

Forward-Looking Statements

This press release contains forward-looking statements that are based on Larimar s management s beliefs and assumptions and on information

currently available to management. All statements contained in this release other than statements of historical fact are forward-looking statements, including but not limited to statements regarding Larimar s expectations regarding its ability

to resolve the clinical hold imposed by the FDA related to CTI-1601, Larimar s ability to develop and commercialize CTI-1601 and other planned product candidates,

research and development efforts, and other matters regarding Larimar s business strategies, use of capital, results of operations and financial position, and plans and objectives for future

In some cases, you can identify forward-looking statements by the words may, will, could,

would, should, expect, intend, plan, anticipate, believe, estimate, predict, project, potential,

continue, ongoing or the negative of these terms or other comparable terminology, although not all forward-looking statements contain these words. These statements involve risks, uncertainties and other factors that may cause

actual results, performance, or achievements to be materially different from the information expressed or implied by these forward-looking statements. These risks, uncertainties and other factors include, among others, Larimar s ability to

successfully engage with the FDA and satisfactorily respond to requests from the FDA for further information and data regarding CTI-1601, the timing and outcome of Larimar s planned interactions with the

FDA concerning the clinical hold on CTI-1601, the success, cost and timing of Larimar s product development activities, nonclinical studies and clinical trials, including

CTI-1601 clinical milestones; that clinical trial results may differ from final clinical trial results, that earlier non-clinical and clinical data and testing of CTI-1601 may not be predictive of the results or success of clinical trials, and assessments; the ongoing impact of the COVID-19 pandemic on Larimar s future clinical

trials, manufacturing, regulatory and nonclinical study timelines, ability to raise additional capital and general economic conditions; Larimar s ability to optimize and scale CTI-1601 s

manufacturing process; Larimar s ability to obtain regulatory approval for CTI-1601 and future product candidates; Larimar s ability to develop sales and marketing capabilities, whether alone or with

potential future collaborators, and to successfully commercialize any approved product candidates; Larimar s ability to raise the necessary capital to conduct its product development activities; and other risks described in the filings made

by Larimar with the Securities and Exchange Commission (SEC), including but not limited to Larimar s periodic reports, including the annual report on Form 10-K, quarterly reports on

Form 10-Q and current reports on Form 8-K, filed with or furnished to the SEC and available at www.sec.gov. These forward-looking statements are

based on a combination of facts and factors currently known by Larimar and its projections of the future, about which it cannot be certain. As a result, the forward-looking statements may not prove to be accurate. The forward-looking

statements in this press release represent views as of the date hereof. Larimar undertakes no obligation to update any forward-looking statements for any reason, except as required by law.

Larimar Therapeutics, Inc.

Consolidated Balance Sheet

Larimar Therapeutics, Inc.

Consolidated Statements of Operations

(In thousands, except share and per share data)