Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
Functional amelioration of murine galactosialidosis by genetically modified bone marrow hematopoietic progenitor cells by Thasia Leimig, Linda Mann, Maria del Pilar Martin, Erik Bonten, Derek Persons, James Knowles, James A. Allay, John Cunningham, Arthur W. Nienhuis, Richard Smeyne, and Alessandra d'Azzo Blood Volume 99(9):3169-3178 May 1, 2002 ©2002 by American Society of Hematology Retrovirally transduced PPCA−/− BM cells restore cathepsin A activity in transplanted PPCA−/− recipients.(A) Schematic diagram of the retroviral bicistronic construct encoding the human PPCA cDNA. Thasia Leimig et al. Blood 2002;99:3169-3178 ©2002 by American Society of Hematology Histology of systemic organs from BM-transplanted GS mice.Organs from PPCA −/− mice transplanted with total −/−BM transduced with the MSCV-PPCA (BMT-PPCA) retrovirus were isolated at different time points after treatment. Thasia Leimig et al. Blood 2002;99:3169-3178 ©2002 by American Society of Hematology Immunostaining of tissue sections from BM-transplanted GS mice with α-32 and α-GFP antibodies.Numerous human PPCA-expressing cells were detected by immunostaining with α-32 antibody, monospecific for the human protein (left panels). Thasia Leimig et al. Blood 2002;99:3169-3178 ©2002 by American Society of Hematology Histology of 4 regions of the brain from BM-transplanted GS mice.Hematoxylin and eosin staining of the choroid plexus (CP), the lateral geniculate nucleus (LGN), the amygdala (AMG), and the deep cerebellar nucleus (CbN) isolated from BMT-PPCA–treated GS mou... Thasia Leimig et al. Blood 2002;99:3169-3178 ©2002 by American Society of Hematology Immunostaining of 4 brain regions from BM-transplanted GS mice with α-32 and α-GFP antibodies.α-32 immunostaining of brain sections derived from BMT-PPCA–transplanted mice at 9 months after treatment revealed numerous PPCA+ endothelial cells, perivascular m... Thasia Leimig et al. Blood 2002;99:3169-3178 ©2002 by American Society of Hematology PEP19 staining of cerebellar sections from BM-transplanted GS mice.Serial sections of the cerebellum from a 9-month-old GS mouse transplanted with MSCV-PPCA–marked BM cells were stained with anti-PEP19 antibody. Thasia Leimig et al. Blood 2002;99:3169-3178 ©2002 by American Society of Hematology Purkinje cell counts in BM-transplanted GS mice.Purkinje cells were counted in recipient mice at 3 and 9 months after treatment and compared to 3- and 9-month-oldPPCA −/− mice and agematched controls. Thasia Leimig et al. Blood 2002;99:3169-3178 ©2002 by American Society of Hematology