Protara Therapeutics Provides Comparability and U.S. Regulatory Updates for TARA-002 Supporting Advancement in Oncology and Rare Disease Indications - Company Demonstrates Initial Comparability Between TARA-002 and OK-43

Protara Therapeutics Provides Comparability

and U.S. Regulatory Updates for TARA-002 Supporting Advancement in Oncology and Rare Disease Indications

- Company Demonstrates Initial Comparability

Between TARA-002 and OK-432, Advancing to Final GMP Comparability Runs -

- Reaches Alignment with FDA on Development

Path Forward for TARA-002 in Non-Muscle Invasive Bladder Cancer; Company Plans to Initiate Clinical Trials in 2021 -

- Company Expects to Request Meeting

with FDA by Year End to Discuss Path to BLA for TARA-002 in Lymphatic Malformations -

NEW YORK, September 8, 2020 - Protara Therapeutics,

Inc. (Nasdaq: TARA), a clinical-stage company developing transformative therapies for the treatment of cancer and rare diseases

with significant unmet needs, today announced its development plans for TARA-002 in both non-muscle invasive bladder cancer (NMIBC)

and Lymphatic Malformations (LMs) following recent interactions with the U.S. Food and Drug Administration (FDA). TARA-002 is the

Company's lead investigational cell therapy based on the broad immunopotentiator OK-432, which is approved in Japan and Taiwan

for the treatment of LMs and multiple oncologic indications.

"The outcome of our recent interaction

with the FDA represents a significant milestone for the TARA-002 program, confirming initial comparability between TARA-002 and

OK-432 and expanding the potential market opportunity of TARA-002 beyond LMs to include a potentially significant oncology indication,"

said Jesse Shefferman, Chief Executive Officer of Protara Therapeutics. "Importantly, we believe that we have identified

an efficient path forward that allows us to address the population of patients suffering from NMIBC who are lacking alternative

therapeutic options. Patients suffering from NMIBC have limited approved treatment options, and with the current standard-of-care

facing a historical long-term supply shortage, there is a dire need for novel and effective therapies for these patients."

Mr. Shefferman added, "In addition

to the opportunity in NMIBC, as we near completion of our IND update and make progress toward finalizing GMP comparability of TARA-002

to OK-432, we continue to expect to request a meeting with the FDA by year end to discuss a potential near-term path to a BLA filing

for TARA-002 in LMs. TARA-002 was recently granted a Rare Pediatric Disease Designation for LMs, a rare pediatric indication with

an urgent need for new therapeutic approaches."

FDA Confirmed Initial Comparability

Between TARA-002 and OK-432; Final GMP Comparability Expected to be Completed in Mid-2021

Following a recent pre-Investigational

New Drug (IND) engagement with the Office of Tissues and Advanced Therapies division of the Center for Biologics Evaluation and

Research (CBER), the FDA agreed that Protara has successfully demonstrated initial manufacturing comparability between TARA-002

and OK-432 and that the Company was on track with its plans to conduct three large-scale batch runs to confirm comparability. Good

Manufacturing Practice (GMP) scale up is currently in process and the Company will initiate GMP comparability runs with an expected

completion date in mid-2021.

Clinical Development Path in NMIBC

In addition, the Company reached alignment

with the FDA on a proposed clinical development plan to evaluate TARA-002 in patients with NMIBC. Advancement into the clinic will

be supported by existing and ongoing non-clinical studies as well as the historical safety and efficacy data for OK-432.

Subject to the successful completion of

select non-clinical studies to characterize local toxicity of intravesical administration of TARA-002 as well as acceptance

of an IND filing, the Company plans to commence a Phase 1 study in 2021 to assess the safety and tolerability of TARA-002 in

patients with NMIBC, including patients with carcinoma in situ (CIS), with results expected in 2022.

The Phase 2 development program, which

Protara plans to commence in 2022, is expected to include NMIBC patients with CIS +/- Ta and/or T1 papillary tumors and high-grade

Ta and/or T1 papillary tumors without CIS.

Regulatory Path in LMs

Protara plans to request a meeting with

the FDA Division of Vaccines and Related Products Applications by year end to discuss the regulatory path for TARA-002 in LMs.

The Company plans to utilize the robust dataset for OK-432 in LMs to support a Biological License Application (BLA) filing for

TARA-002 in LMs. In a randomized, Phase 2 clinical trial of OK-432 in LMs conducted in the U.S., 68% of patients treated with OK-432

(>90% pediatric) in the immediate treatment group experienced a complete or substantial response. Long-term control of LMs was

favorable, with more than 90% of patients treated with OK-432 having no regrowth over a median follow-up period of approximately

three years following treatment.

TARA-002 is an investigational cell therapy

in development for the treatment of lymphatic malformations (LMs) and non-muscle invasive bladder cancer (NMIBC). TARA-002 was

developed from the same master cell bank of genetically distinct group A Streptococcus pyogenes as OK-432, a broad immunopotentiator

marketed as Picibanil in Japan and Taiwan by Chugai Pharmaceutical Co., Ltd. Protara successfully demonstrated

initial manufacturing comparability between TARA-002 and OK-432.

When TARA-002 is administered, it is hypothesized

that innate and adaptive immune cells within the cyst or tumor are activated and produce a strong immune cascade. Neutrophils,

monocytes and lymphocytes infiltrate the abnormal cells and various cytokines, including interleukins IL-6, IL-8, IL-12, interferon

(IFN)-gamma, tumor necrosis factor (TNF)-alpha, and vascular endothelial growth factor (VEGF) are secreted by immune cells to induce

a strong local inflammatory reaction and destroy the abnormal cells. TARA-002 has been granted Rare Pediatric Disease Designation

by the U.S. Food and Drug Administration for the LMs indication.

About Non-Muscle Invasive Bladder Cancer

Bladder cancer is the 6th most common cancer

in the United States, with non-muscle invasive bladder cancer (NMIBC) representing approximately 80% of bladder cancer diagnoses.

Approximately 65,000 patients are diagnosed with NMIBC in the United States each year. NMIBC is cancer found in the tissue that

lines the inner surface of the bladder that has not spread into the bladder muscle. The current standard of care for high-grade

NMIBC includes intravesical Bacillus Calmette-Guerin (BCG), which has been the subject of multiple global supply shortages in the

About Lymphatic Malformations

Lymphatic malformations (LMs) are rare,

congenital malformations of lymphatic vessels resulting in the failure of these structures to connect or drain into the venous

system. Most LMs present in the head and neck region and are diagnosed in early childhood during the period of active lymphatic

growth, with more than 50% detected at birth and 90% diagnosed before the age of 2 years. The most common morbidities and serious

manifestations of the disease include compression of the upper aerodigestive tract, including airway obstruction requiring intubation

and possible tracheostomy dependence; intralesional bleeding; impingement on critical structures, including nerves, vessels, lymphatics;

recurrent infection, and cosmetic and other functional disabilities.

About Protara Therapeutics, Inc.

Protara is committed to identifying and

advancing transformative therapies for people with cancer and rare diseases with limited treatment options. Protara's portfolio

includes its lead program, TARA-002, an investigational cell-based therapy being developed for the treatment of non-muscle invasive

bladder cancer and lymphatic malformations, and IV Choline Chloride, an investigational phospholipid substrate replacement therapy

for the treatment of intestinal failure-associated liver disease. For more information, visit www.protaratx.com.

Forward-Looking Statements

Statements contained in this press release

regarding matters that are not historical facts are "forward looking statements" within the meaning of the Private Securities

Litigation Reform Act of 1995. Protara may, in some cases, use terms such as "predicts," "believes," "potential,"

"proposed," "continue," "designed," "estimates," "anticipates," "expects,"

"plans," "intends," "may," "could," "might," "will," "should"

or other words or expressions referencing future events, conditions or circumstances that convey uncertainty of future events or

outcomes to identify these forward-looking statements. Such forward-looking statements include but are not limited to, statements

regarding Protara's intentions, beliefs, projections, outlook, analyses or current expectations concerning, among other things:

statements regarding Protara's business strategy, Protara's manufacturing and development plans for its product candidates

and related interactions with the FDA. Because such statements are subject to risks and uncertainties, actual results may differ

materially from those expressed or implied by such forward-looking statements. Factors that contribute to the uncertain nature

of the forward-looking statements include risks and uncertainties associated with: Protara's development programs, including

the initiation and completion of non-clinical studies and clinical trials and the timing of required filings with the FDA and other

regulatory agencies; the impact of the COVID-19 pandemic on Protara's business, clinical supply chain, clinical trials and

the global economy; general market conditions; changes in the competitive landscape; changes in Protara's strategic and commercial