Calcium homeostasis in lysosomal storage diseases

International Journal of Clinical Pharmacology and Therapeutics

Volumn 48, Issue SUPPL. 1, 2010, Pages

  Futerman, A H ^a  

^a WEIZMANN INSTITUTE OF SCIENCE   (Israel)

Author keywords

[No Author keywords available]

Indexed keywords

CCAAT ENHANCER BINDING PROTEIN; GANGLIOSIDE GM1; GANGLIOSIDE GM2; GLUCOSE REGULATED PROTEIN; GLUCOSE REGULATED PROTEIN 58; GLUCOSYLCERAMIDE; MESSENGER RNA; RYANODINE RECEPTOR; SARCOPLASMIC RETICULUM CALCIUM TRANSPORTING ADENOSINE TRIPHOSPHATASE; UNCLASSIFIED DRUG; X BOX BINDING PROTEIN 1;

APOPTOSIS; CALCIUM HOMEOSTASIS; CALCIUM TRANSPORT; CONFERENCE PAPER; ENDOPLASMIC RETICULUM STRESS; GAUCHER DISEASE; LYSOSOME STORAGE DISEASE; NERVE DEGENERATION; NIEMANN PICK DISEASE; NONHUMAN; PATHOGENESIS; PURKINJE CELL; SANDHOFF DISEASE;

EID: 77949474575     PISSN: 09461965     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Conference Paper

References (12)

1. Farfel-Becker T, Vitner E, Dekel H, Leshem N, Enquist IB, Karlsson S et al. No evidence for activation of the unfolded protein response in neuronopathic models of Gaucher disease. Hum Mol Genet. 2009; 18: 1482-1488.
2. Futerman AH, van Meer G. The cell biology of lysosomal storage disorders. Nat Rev Mol Cell Biol. 2004; 5: 554-565.
3. Ginzburg L, Kacher Y, Futerman AH. The pathogenesis of glycosphingolipid storage disorders. Semin Cell Dev Biol. 2004; 15: 417-431.
4. Ginzburg L, Futerman AH. Defective calcium homeostasis in the cerebellum in a mouse model of Niemann-Pick A disease. J Neurochem. 2005; 95: 1619-1928.
5. 2+-ATPase: relevance to gangliosidoses. J Neurochem. 2008; 104: 140-146.
6. Kacher Y, Futerman AH. Genetic diseases of sphingolipid metabolism: pathological mechanisms and therapeutic options. FEBS Lett. 2006; 580: 5510-5517.
7. Korkotian E, Schwarz A, Pelled D, Schwarzmann G, Segal M, Futerman AH. Elevation of intracellular glucosylceramide levels results in an increase in endoplasmic reticulum density and in functional calcium stores in cultured neurons. J Biol Chem. 1999; 274: 21673-21678.
8. Pelled D, Shogomori H, Futerman AH. The increased sensitivity of neurons with elevated glucocerebroside to neurotoxic agents can be reversed by imiglucerase. J Inherit Metab Dis. 2000; 23: 175-184.
9. 2+-ATPase in a mouse model of Sandhoff disease and prevention by treatment with N-butyldeoxynojirimycin. J Biol Chem. 2003; 278: 29496-29501.
10. Pelled D, Trajkovic-Bodennec S, Lloyd-Evans E, Sidransky E, Schiffmann R, Futerman AH. Enhanced calcium release in the acute neuronopathic form of Gaucher disease. Neurobiol Dis. 2005; 18: 83-88.
11. Raas-Rothschild A, Pankova-Kholmyansky I, Kacher Y, Futerman AH. Glycosphingolipidoses: beyond the enzymatic defect. Glycoconj J. 2004; 21: 295-304.
12. Wei H, Kim SJ, Zhang Z, Tsai PC, Wisniewski KE, Mukherjee AB. ER and oxidative stresses are common mediators of apoptosis in both neurodegenerative and non-neurodegenerative lysosomal storage disorders and are alleviated by chemical chaperones. Hum Mol Genet. 2008; 17: 469-477.