G sub(M1)-gangliosidosis in American black bears: Clinical, pathological, biochemical and molecular genetic characterization

• Published in: Molecular genetics and metabolism Vol. 111; no. 4; pp. 513 - 521
• Main Authors: Muthupalani, Sureshkumar, Torres, Paola A, Wang, Betty C, Zeng, Bai Jin, Eaton, Samuel, Erdelyi, Ildiko, Ducore, Rebecca, Maganti, Rajanikarath, Keating, John, Perry, Bain J, Tseng, Florina S, Waliszewski, Nicole, Pokras, Mark, Causey, Robert, Seger, Rita, March, Philip, Tidwell, Amy, Pfannl, Rolf, Seyfried, Thomas, Kolodny, Edwin H, Alroy, Joseph
• Format: Journal Article
• Language: English
• Published: 01.04.2014
• Subjects: Ursus americanus

G sub(M1)-gangliosidosis is a rare progressive neurodegenerative disorder due to an autosomal recessively inherited deficiency of lysosomal beta -galactosidase. We have identified seven American black bears (Ursus americanus) found in the Northeast United States suffering from G sub(M1)-gangliosidos...