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5 results on '"Biswas, Sunita"'

1. Large-Scale Phenotyping of an Accurate Genetic Mouse Model of JNCL Identifies Novel Early Pathology Outside the Central Nervous System

Author

Staropoli, John F., primary, Haliw, Larissa, additional, Biswas, Sunita, additional, Garrett, Lillian, additional, Hölter, Sabine M., additional, Becker, Lore, additional, Skosyrski, Sergej, additional, Da Silva-Buttkus, Patricia, additional, Calzada-Wack, Julia, additional, Neff, Frauke, additional, Rathkolb, Birgit, additional, Rozman, Jan, additional, Schrewe, Anja, additional, Adler, Thure, additional, Puk, Oliver, additional, Sun, Minxuan, additional, Favor, Jack, additional, Racz, Ildikó, additional, Bekeredjian, Raffi, additional, Busch, Dirk H., additional, Graw, Jochen, additional, Klingenspor, Martin, additional, Klopstock, Thomas, additional, Wolf, Eckhard, additional, Wurst, Wolfgang, additional, Zimmer, Andreas, additional, Lopez, Edith, additional, Harati, Hayat, additional, Hill, Eric, additional, Krause, Daniela S., additional, Guide, Jolene, additional, Dragileva, Ella, additional, Gale, Evan, additional, Wheeler, Vanessa C., additional, Boustany, Rose-Mary, additional, Brown, Diane E., additional, Breton, Sylvie, additional, Ruether, Klaus, additional, Gailus-Durner, Valérie, additional, Fuchs, Helmut, additional, de Angelis, Martin Hrabě, additional, and Cotman, Susan L., additional

Published
2012
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5. Distinct Early Molecular Responses to Mutations Causing vLINCL and JNCL Presage ATP Synthase Subunit C Accumulation in Cerebellar Cells.

Author

Yi Cao, Staropoli, John F., Biswas, Sunita, Espinola, Janice A., MacDonald, Marcy E., Jong-Min Lee, and Cotman, Susan L.

Subjects
GENETIC mutation, ADENOSINE triphosphatase, ORGANELLES, NEURONAL ceroid-lipofuscinosis, CELL physiology, CELLULAR aging, GENETIC regulation, GENE expression
Abstract

Variant late-infantile neuronal ceroid lipofuscinosis (vLINCL), caused by CLN6 mutation, and juvenile neuronal ceroid lipofuscinosis (JNCL), caused by CLN3 mutation, share clinical and pathological features, including lysosomal accumulation of mitochondrial ATP synthase subunit c, but the unrelated CLN6 and CLN3 genes may initiate disease via similar or distinct cellular processes. To gain insight into the NCL pathways, we established murine wild-type and CbCln6nclf/nclf cerebellar cells and compared them to wild-type and CbCln3Λex7/8/Λex7/8 cerebellar cells. CbCln6nclf/nclf cells and CbCln3Λex7/8/Λex7/8 cells both displayed abnormally elongated mitochondria and reduced cellular ATP levels and, as cells aged to confluence, exhibited accumulation of subunit c protein in Lamp 1-positive organelles. However, at sub-confluence, endoplasmic reticulum PDI immunostain was decreased only in CbCln6nclf/nclf cells, while fluid-phase endocytosis and LysoTrackerH labeled vesicles were decreased in both CbCln6nclf/nclf and CbCln3Λex7/8/Λex7/8 cells, though only the latter cells exhibited abnormal vesicle subcellular distribution. Furthermore, unbiased gene expression analyses revealed only partial overlap in the cerebellar cell genes and pathways that were altered by the Cln3Λex7/8 and Cln6nclf mutations. Thus, these data support the hypothesis that CLN6 and CLN3 mutations trigger distinct processes that converge on a shared pathway, which is responsible for proper subunit c protein turnover and neuronal cell survival. [ABSTRACT FROM AUTHOR]

Published
2011
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