Clinically in urgent need of a new drug for rare diseases, Aga glycoenzyme alpha, has been approved in China to treat Fabre disease.

On August 28th, Baisseng Biotech submitted in China an application for the listing of a strong solution for Aga glycosin alpha injection, which was approved for listing by the State Drug Administration, and the drug belonged to the "second batch of clinically urgent need for overseas drug use", the adaptive disease is Fabre disease.
a enzyme replacement therapy developed by Shire (Takeda) and approved by the European Union in March 2001 with sales of $490 million in 2018.
Fabre disease is a congenital metabolic disease caused by the defects of the X chain gene, which manifests it as a significant decrease or loss of alpha-semi-lactose glycosidease A activity in the patient's body, resulting in the pathological accumulation of glycolipids in the lysosomes of a variety of tissue cells.
the disease originated in the fetus and gradually worsened with the patient's life, causing heart, kidney and other important organ failure.
2018, the disease is included in the country's first list of rare diseases, is a serious life-threatening disease and there is no effective treatment in the country.
December 2019, Sanofi's injection of agaglyzyme beta (commodity name: Fabzan) was approved in China, the first drug approved in China to treat Fabre disease.
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