Adriana Montaño, Ph.D.
Assistant Research Professor
Department of Pediatrics
Enzyme replacement therapy, gene therapy, and other treatments for mucopolysaccharidosis type IVA or Morquio Disease.
Office: DRC, Room 311
Phone: (314) 977-9103
Ph.D., 2001, Gifu University School of Medicine
We are working on developing a registry and growth charts for patients with MPS IVA and are in pre-clinical trials for enzyme replacement therapy for patients affected by MPS IVA. We are also developing a bone targeting system for treatment of MPS disorders, substrate reudction therapy for MPSs, and gene therapy for MPS IVA. Other areas of interest are characterization of hyaluronidases, the molecular evolution of glycosaminoglycans and peptidoglycan receptor proteins, and chondroitin sulfate metabolism.
- Clinical course of sly syndrome.
Montano AM, Lock-Hock N, et al. J Med Genet. (2016) Feb 23 [Epub ahead of print].
- Impact of enzyme replacement therapy and hematopoietic stem cell transplantation in patients with Morquio A syndrome.
Tomatsu S, Sawamoto K, et al. Drugs Des Devel Ther. (2015) 9:1937-1953.
- Di-sulfated keratan sulfate as a novel biomarker for mucopolysaccharidosis II, IVA, and IVB.
Shimada T, Tomatsu S, et al. JIMD Rep. (2015) 21:1-13.
- Therapies for the bone in mucopolysaccharidoses.
Tomatsu S, Almeciga-Diaz CJ, et al. Mol Genet Metab. (2015) 114(2):94-109.
- Activities of daily living in patients with Hunter syndrome: Impact of enzyme replacement therapy and hematopoietic stem cell transplantation.
Tanjuakio J, Suzuki Y, et al. Mol Genet Metab. (2015) 114(2):161-169.