Early changes in the Apparent Diffusion Coefficient (ADC) in a mouse model of sandhoff's disease occur prior to disease symptoms and behavioral deficits

Magnetic Resonance in Medicine

Volumn 62, Issue 5, 2009, Pages 1175-1184

  Lingyun, Hu ^a   Yingying, Sun ^a   Villasana, Laura E ^c   Paylor, Richard ^b   Klann, Eric ^d   Pautler, Robia G ^a,b  

^a BCM: 335 ^*  (United States)

^b BAYLOR COLLEGE OF MEDICINE   (United States)

^c OREGON HEALTH AND SCIENCE UNIVERSITY   (United States)

^d NEW YORK UNIVERSITY   (United States)

Author keywords

ADC; Diffusion; Gangliosides; Hex ; Lysosomal storage disease; Mouse; MRI; Sandhoff's disease

Indexed keywords

BRAIN; DIFFUSION; DIGITAL STORAGE; MAGNETIC RESONANCE IMAGING;

APPARENT DIFFUSION COEFFICIENT; BRAIN REGIONS; CELL SWELLING; DIFFUSION OF WATER; DIFFUSION WEIGHTED IMAGING; DISEASE SYMPTOMS; GANGLIOSIDES; MOUSE;

SURFACE DIFFUSION;

GANGLIOSIDE GM2;

ADOLESCENT; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BEHAVIOR CHANGE; CELL SWELLING; CONTROLLED STUDY; DIFFUSION COEFFICIENT; DIFFUSION WEIGHTED IMAGING; EXPLORATORY BEHAVIOR; FEMALE; LYSOSOME STORAGE DISEASE; MALE; MAZE TEST; MOUSE; NONHUMAN; OPEN FIELD BEHAVIOR; SANDHOFF DISEASE; SYMPTOMATOLOGY;

EID: 70449440600     PISSN: 07403194     EISSN: 15222594     Source Type: Journal    
DOI: 10.1002/mrm.22138     Document Type: Article

References (21)

1. Brooksbank BW, McGovern J. Gangliosides in the brain in adult Down's syndrome and Alzheimer's disease. Mol Chem Neuropathol 1989;11:143-156. (Pubitemid 20322833)
2. Ariga T, Kobayashi K, Hasegawa A, Kiso M, Ishida H, Miyatake T. Characterization of high-affinity binding between gangliosides and amyloid beta-protein. Arch Biochem Biophys 2001;388:225-230. (Pubitemid 32911613)
3. Ledeen RW, Sclafani JR. New strategies for detection and resolution of minor gangliosides as applied to brain fuco-gangliosides. Methods Enzymol 1982;83:139-189.
4. Svennerholm L. Biological significance of gangliosides' cellular and pathological aspects of glycoconjugate metabolism. INSERM 1984;126:21-44.
5. van Echten G, Sandhoff K. Modulation of ganglioside biosynthesis in primary cultured neurons. J Neurochem 1989;52:207-214. (Pubitemid 19023370)
6. Kojima N, Hakomori S. Cell adhesion, spreading, and motility of GM3-expressing cells based on glycolipid-glycolipid interaction. J Biol Chem 1991;266:17552-17558. (Pubitemid 21907973)
7. Karlsson KA. Animal glycosphingolipids as membrane attachment sites for bacteria. Annu Rev Biochem 1989;58:309-350.
8. Phillips ML, Nudelman E, Gaeta FC, Perez M, Singhal AK, Hakomori S, Paulson JC. ELAM-1 mediates cell adhesion by recognition of a carbohydrate ligand, sialyl-Lex. Science 1990;250:1130-1132. (Pubitemid 120031820)
9. Jeyakumar M, Butters TD, Dwek RA, Platt FM. Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis. Neuropathol Appl Neurobiol 2002;28:343-357. (Pubitemid 35279069)
10. Jeyakumar M, Dwek RA, Butters TD, Platt FM. Storage solutions: treating lysosomal disorders of the brain. Nat Rev Neurosci 2005;6:713-725. (Pubitemid 44773245)
11. Neufeld EF. Natural history and inherited disorders of a lysosomal enzyme, beta-hexosaminidase. J Biol Chem 1989;264:10927-10930.
12. Phaneuf D, Wakamatsu N, Huang JQ, Borowski A, Peterson AC, Fortunato SR, Ritter G, Igdoura SA, Morales CR, Benoit G, Akerman BR, Leclerc D, Hanai N, Marth JD, Trasler JM, Gravel RA. Dramatically different phenotypes in mouse models of human Tay-Sachs and Sandhoff diseases. Hum Mol Genet 1996;5:1-14. (Pubitemid 26018268)
13. Sango K, Yamanaka S, Hoffmann A, Okuda Y, Grinberg A, Westphal H, McDonald MP, Crawley JN, Sandhoff K, Suzuki K, Proia RL. Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism. Nat Genet 1995;11:170-176.
14. Hittmair K, Wimberger D, Bernert G, Mallek R, Schindler EG. MRI in a case of Sandhoff's disease. Neuroradiology 1996;38(suppl 1):S178-180.
15. Koelfen W, Freund M, Jaschke W, Koenig S, Schultze C. GM-2 gangliosidosis (Sandhoff's disease): two year follow-up by MRI. Neuroradiology 1994;36:152-154. (Pubitemid 24040271)
16. Yun YM, Lee SN. A case report of Sandhoff disease. Korean J Ophthalmol 2005;19:68-72.
17. Yuksel A, Yalcinkaya C, Islak C, Gunduz E, Seven M. Neuroimaging findings of four patients with Sandhoff disease. Pediatr Neurol 1999;21:562-565. (Pubitemid 29366825)
18. Caliskan M, Ozmen M, Beck M, Apak S. Thalamic hyperdensity: is it a diagnostic marker for Sandhoff disease? Brain Dev 1993;15:387-388.
19. Taniike M, Yamanaka S, Proia RL, Langaman C, Bone-Turrentine T, Suzuki K. Neuropathology of mice with targeted disruption of Hexa gene, a model of Tay-Sachs disease. Acta Neuropathol 1995;89:296-304.
20. Yamanaka S, Johnson MD, Grinberg A, Westphal H, Crawley JN, Taniike M, Suzuki K, Proia RL. Targeted disruption of the Hexa gene results in mice with biochemical and pathologic features of Tay-Sachs disease. Proc Natl Acad Sci U|S|A 1994;91:9975-9979. (Pubitemid 24311931)
21. Yamanaka S, Johnson ON, Norflus F, Boles DJ, Proia RL. Structure and expression of the mouse beta-hexosaminidase genes, Hexa and Hexb Genomics 1994;21:588-596.