8-K

UNITED STATES

SECURITIES AND EXCHANGE COMMISSION

Washington, D.C. 20549

FORM 8-K

CURRENT REPORT

Pursuant to Section 13 or 15(d)

of the Securities Exchange Act of 1934

Date of Report (Date of earliest event reported): September 4, 2018

AEGLEA BIOTHERAPEUTICS, INC.

(Exact name of registrant as specified in its charter)

Delaware	001-37722	46-4312787
(State or other jurisdiction of incorporation)	(Commission File Number)	(IRS Employer Identification No.)

901 S. MoPac Expressway Barton Oaks Plaza One Suite 250 Austin, TX	78746
(Address of principal executive offices)	(Zip Code)

(512) 942-2935

(Registrant’s telephone number, including area code)

(Former name or former address, if changed since last report)

Check the appropriate box below if the Form 8-K filing is intended to simultaneously satisfy the filing obligation of the registrant under any of the following provisions:

☐ Written communications pursuant to Rule 425 under the Securities Act (17 CFR 230.425)

☐ Soliciting material pursuant to Rule 14a-12 under the Exchange Act (17 CFR 240.14a-12)

☐ Pre-commencement communications pursuant to Rule 14d-2(b) under the Exchange Act (17 CFR 240.14d-2(b))

☐ Pre-commencement communications pursuant to Rule 13e-4(c) under the Exchange Act (17 CFR 240.13e-4(c))

Indicate by check mark whether the registrant is an emerging growth company as defined in Rule 405 of the Securities Act of 1933 (§230.405 of this chapter) or Rule 12b-2 of the Securities Exchange Act of 1934 (§240.12b-2 of this chapter).

Emerging growth company ☒

If an emerging growth company, indicate by check mark if the registrant has elected not to use the extended transition period for complying with any new or revised financial accounting standards provided pursuant to Section 13(a) of the Exchange Act. ☒

Item 7.01 Regulation FD Disclosure.

On September 4, 2018, Aeglea BioTherapeutics, Inc. (the “Company”) issued a press release announcing an enrollment status update and new interim Phase 1/2 clinical trial data demonstrating clinically relevant treatment effects in patients with Arginase 1 Deficiency, which will be presented in poster format at the 2018 Society for the Study of Inborn Errors of Metabolism (SSIEM) Annual Symposium in Athens, Greece, being held September 4-7, 2018. A copy of the press release and presentation poster are attached as Exhibits 99.1 and 99.2 to this report, respectively. The presentation poster will also be available on the Company’s website in the Events & Presentations section at www.aegleabio.com.

The information furnished with this report, including Exhibits 99.1 and 99.2, shall not be deemed “filed” for purposes of Section 18 of the Securities Exchange Act of 1934, as amended (“Exchange Act”), or otherwise subject to the liabilities of that section, nor shall it be deemed incorporated by reference into any other filing under the Securities Act of 1933, as amended, or the Exchange Act, except as expressly set forth by specific reference in such a filing.

Item 9.01Financial Statements and Exhibits.

(d) Exhibits

Exhibit Number	Description
99.1	Press Release issued by Aeglea BioTherapeutics, Inc. on September 4, 2018
99.2	Presentation Poster

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SIGNATURE

Pursuant to the requirements of the Securities Exchange Act of 1934, the registrant has duly caused this report to be signed on its behalf by the undersigned hereunto duly authorized.

	AEGLEA BIOTHERAPEUTICS, INC.
Date: September 4, 2018	By:	/s/ Charles N. York II
		Charles N. York II
		Chief Financial Officer

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EX-99.1

Exhibit 99.1

Aeglea BioTherapeutics Announces Positive Interim Clinical Data and Completion of Enrollment for Ongoing Phase 1/2 Trial of Pegzilarginase in Patients with Arginase 1 Deficiency

Exceeded Enrollment Target with 15 Patients in Phase 1/2 Trial

Additional Interim Data to be Presented at ASHG Conference in October 2018

Company Plans to Announce Pivotal Trial Design in Q4 2018 and Initiate Pivotal Trial in 1H 2019

Company to Host Clinical Update Conference Call Today at 8:30 a.m. ET

Austin, Texas, September 4, 2018 - Aeglea BioTherapeutics, Inc. (NASDAQ: AGLE), a clinical-stage biotechnology company that designs and develops innovative human enzyme therapeutics for patients with rare genetic diseases and cancer, today announced completion of enrollment and additional clinical data at the 2018 Society for the Study of Inborn Errors of Metabolism (SSIEM) Annual Symposium from its ongoing Phase 1/2 trial of pegzilarginase, its lead investigational therapy, in patients with the rare genetic disease Arginase 1 Deficiency (ARG1-D).

“I am thrilled that we exceeded our recruitment target in this Phase 1/2 trial of pegzilarginase by five patients, as enrollment completion is an important milestone for patients with ARG1-D,” said Anthony G. Quinn, M.B Ch.B, Ph.D., president and chief executive officer of Aeglea. “We are pleased to confirm our previous guidance of announcing the design of the first pivotal clinical trial for patients with ARG1-D in the fourth quarter of 2018 and initiating the pivotal trial in the first half of 2019.”

“Given the challenges of lowering arginine levels with current approaches, it is very encouraging to see the marked reductions in plasma arginine in our patient following treatment with pegzilarginase,” said George Diaz, M.D., Ph.D, professor and chief, Division of Medical Genetics, Icahn School of Medicine at Mount Sinai, and co-author on the presentation. “In addition, it is very exciting that we are seeing evidence of an impact on important disease manifestations with better walking, improved posture, and enhanced alertness.”

Highlights of the SSIEM presentation, entitled "Improvements in Arginase 1 Deficiency Related Disease Manifestations Following Plasma Arginine Reductions with Pegzilarginase," include the following:

- Administration of pezilarginase resulted in marked reductions in plasma arginine and related guanidino compounds (GCs);

- Clinical improvements in one or more instruments of neuromotor function in all three patients completing eight weeks of repeat dose administration;

- Pegzilarginase was generally safe and well tolerated; most treatment-related adverse events (AEs) were mild and all were resolved;

- No marked or sustained increase in ADA titers in patients exposed to pegzilarginase and initial evidence of rapid tolerization in patients with low titer ADAs.

The Company plans to present new interim clinical data for the Phase 1/2 trial at the 2018 American Society of Human Genetics (ASHG) Conference in October.

Conference Call & Webcast Details

Aeglea will hold a clinical update conference call today, Tuesday, September 4, 2018 at 8:30 a.m. ET. To access the live conference call via phone, please dial 1-877-709-8155 (toll free) within the United States, or 1-201-689-8881 internationally. A replay of the call will be available through September 11, 2018 by dialing 1-877-660-6853 within the United States or 1-201-612-7415 internationally. The conference ID is 13678293.

To access the live and archived webcast of the presentation, please visit the Presentations & Events section of the Aeglea BioTherapeutics investor relations website. Please connect to the website at least 15 minutes prior to the presentation to allow for any software download that may be necessary.

About Pegzilarginase in Arginase 1 Deficiency
Pegzilarginase is an enhanced human arginase that enzymatically depletes the amino acid arginine. Aeglea is developing pegzilarginase for the treatment of patients with Arginase 1 Deficiency, a debilitating urea cycle disorder caused by deficiency of a key arginine metabolizing enzyme that leads to severe and progressive hyperargininemia-related neurological abnormalities, hyperammonemia and early mortality. Pegzilarginase is intended for use as an enzyme replacement therapy in patients to reduce elevated blood arginine levels. The Company’s interim Phase 1/2 data demonstrated clinical improvements and rapid and sustained lowering of plasma arginine in Arginase 1 Deficiency patients.

About Aeglea BioTherapeutics
Aeglea is a clinical-stage biotechnology company that designs and develops innovative human enzyme therapeutics for patients with rare genetic diseases and cancer. The Company is developing pegzilarginase, its lead investigational therapy, for the treatment of Arginase 1 Deficiency, as monotherapy in arginine-dependent cancers and in combination with an immune checkpoint inhibitor for small cell lung cancer. In addition, Aeglea has an active research pipeline of other human enzyme-based approaches in both therapeutic areas. For more information, please visit http://aegleabio.com.

Safe Harbor / Forward Looking Statements
This press release contains "forward-looking" statements within the meaning of the safe harbor provisions of the U.S. Private Securities Litigation Reform Act of 1995. Forward-looking statements can be identified by words such as: "anticipate," "intend," "plan," "goal," "seek," "believe," "project," "estimate," "expect," "strategy," "future," "likely," "may," "should," "will" and similar references to future periods. These statements are subject to numerous risks and uncertainties that could cause actual results to differ materially from what we expect. Examples of forward-looking statements include, among others, statements we make regarding the timing and success of our clinical trials and related data, the timing of announcements and updates relating to our clinical trials, trial designs and related data, and the potential therapeutic benefits and economic value of our lead product candidate or other product candidates. Further information on potential risk factors that could affect our business and its financial results are detailed in our most recent Quarterly Report on Form 10-Q for the quarter ended June 30, 2018 filed with the Securities and Exchange Commission (SEC), and other reports as filed with the SEC. We undertake no obligation to publicly update any forward-looking statement, whether written or oral, that may be made from time to time, whether as a result of new information, future developments or otherwise.

Media Contact:
David Calusdian

Sharon Merrill Associates

617.542.5300

AGLE@investorrelations.com

Investor Contact:
Joey Perrone

Director, Finance & Investor Relations

Aeglea BioTherapeutics
investors@aegleabio.com

EX-99.2

Exhibit 99.2