Aeglea Biotherapeutics, Inc., a biopharmaceutical company developing novel treatments for inborn errors of metabolism and therapies targeting tumor metabolism, today announced the closing of a $44 million Series B financing. The financing was led by existing investors Lilly Ventures and Novartis Venture Fund with participation by UT Horizon Fund and new investors, including OrbiMed, Jennison Associates (on behalf of clients), Venrock, RA Capital Management, Rock Springs Capital, Ally Bridge Group and Cowen Investments. The proceeds from the financing will be used to support the continued development of Aeglea's innovative pipeline of engineered human enzymes that target diseases at the extremes of abnormal metabolism. By degrading specific amino acids in circulation, these novel enzymes are able to address inborn errors of metabolism characterized by excess amino acid levels and exploit abnormal amino acid metabolism in tumor cells.

'New treatments for individuals with inborn errors of metabolism or hematologic and solid malignancies are urgently needed, and this financing enables us to advance our lead molecule into the clinical setting as well as pursue the preclinical development of our pipeline products to address significant unmet medical needs'

'New treatments for individuals with inborn errors of metabolism or hematologic and solid malignancies are urgently needed, and this financing enables us to advance our lead molecule into the clinical setting as well as pursue the preclinical development of our pipeline products to address significant unmet medical needs,' said David G. Lowe, Ph.D., co-founder, President and Chief Executive Officer of Aeglea. 'We appreciate the continued strong support of our existing investors and welcome the experience and perspectives of our new investors.'

'We are excited to partner with a company like Aeglea that is working at the intersection of discovery and technology,' said Rajeev Shah, partner at RA Capital Management. 'With an experienced management team, a strong technology platform and four exciting product candidates, we believe Aeglea is poised to have a positive impact on both the treatment of inborn errors of metabolism and cancer.'

Aeglea anticipates that its lead drug candidate, AEB1102 (optimized human Arginase I), will enter Phase I/II clinical proof of concept studies as early as the second half of 2015. AEB1102 is being developed as enzyme replacement therapy in patients with the rare inborn error of metabolism hyperargininemia (HA) that is caused by Arginase I deficiency. This deficiency results in excess levels of arginine in the blood. Patients are predisposed to neurologic symptoms, cognitive deficits, seizures and other clinical signs and symptoms. Left untreated, HA may lead to spasticity, loss of ambulation and severe intellectual disability. The company anticipates that enzyme replacement therapy with AEB1102 will degrade circulating arginine and return blood levels to the normal physiological range. Normalization of arginine levels is expected to slow, and perhaps halt, the progression of disease in these patients. There is currently no effective therapy to treat the underlying cause of HA, which represents a significant unmet medical need for patients suffering from this disease.

Also as early as the second half of 2015, Aeglea plans to initiate Phase I clinical development for AEB1102 in oncology, targeting abnormal tumor metabolism characterized by arginine dependence. Arginine-dependent tumor cells must obtain arginine from the circulation to survive, making them vulnerable to systemic arginine depletion by AEB1102. A variety of studies support the effectiveness of arginine depletion in patients with a variety of cancers, targeting tumors in a way that cannot be achieved with current antibody or small molecule therapeutics. Using established biomarkers, the company anticipates that it will be able to identify cancer patients with tumors that will be sensitive to arginine depletion by AEB1102.

'With its pipeline of optimized drug candidates, this financing enables Aeglea to begin establishing the potential of amino acid degrading enzymes to improve the outcomes associated with these altered metabolic pathways,' said Armen Shanafelt, Ph.D., General Partner at Lilly Ventures and chairman of the board at Aeglea. 'Aeglea is now positioned to become a leader in providing treatments for patients suffering from diseases caused by the extremes of metabolism, as well as treatments that exploit the metabolic vulnerabilities these defects cause in cancer.'

About Aeglea Biotherapeutics

Aeglea was founded in 2013 to develop engineered human enzymes invented in the laboratory of George Georgiou, Ph.D. of The University of Texas at Austin.

Aeglea's pipeline of engineered human enzymes includes AEB1102, which we expect will enter into clinical trials in both hyperargininemia and cancer. In addition, three additional drug candidates are in pre-clinical development: AEB4104, which degrades homocystine, is being evaluated as a potential treatment for the inborn error of metabolism homocystinuria; AEB3103, which degrades cysteine/cysteine, is being evaluated for its ability to increase oxidative stress through glutathione depletion for treatment of hematologic and solid malignancies; and AEB2109, which degrades methionine, is being evaluated for the treatment of solid tumors.

For more information, visit http://aegleabio.com.