|1.||Bongarzone, Ernesto R: 10 articles (01/2015 - 01/2011)|
|2.||Singh, Inderjit: 9 articles (01/2015 - 05/2002)|
|3.||Lopez-Rosas, Aurora: 7 articles (01/2015 - 01/2011)|
|4.||Singh, Avtar K: 7 articles (01/2015 - 05/2002)|
|5.||Wenger, David A: 5 articles (09/2015 - 11/2005)|
|6.||Giri, Shailendra: 5 articles (06/2008 - 05/2002)|
|7.||Givogri, Maria I: 4 articles (04/2014 - 01/2011)|
|8.||Bunnell, Bruce A: 4 articles (02/2014 - 09/2011)|
|9.||Haq, Ehtishamul: 4 articles (09/2008 - 09/2003)|
|10.||Ribatti, Domenico: 3 articles (10/2015 - 09/2013)|
|1.||Globoid Cell Leukodystrophy (Krabbe Disease)
06/15/2005 - "Our results suggest that inhibition of peroxisomal functions and increased free radical production by psychosine may be partly responsible for oligodendrocyte and myelin loss observed in the Krabbe's brain, and that antioxidant therapy may be useful in the treatment of Krabbe's disease."
01/01/1997 - "Previous studies have suggested that psychosine is the causative agent for the pathogenesis of Krabbe's disease. "
01/30/1990 - "Galactosylceramide and galactosylsphingosine loading studies in cultured skin fibroblasts in human and murine globoid cell leukodystrophy."
09/01/2015 - "Measurement of psychosine in dried blood spots--a possible improvement to newborn screening programs for Krabbe disease."
01/28/2015 - "Twitcher and GLD dog muscles had significant capacity to store psychosine, the neurotoxin that accumulates in Krabbe disease. "
|2.||Demyelinating Diseases (Demyelinating Disease)
09/01/2007 - "In this study we evaluated in vitro the cytokine profile of KD patients and the effect of psychosine, the toxic metabolite which plays a role in the demyelination process in these patients. "
09/01/2015 - "Humanized GALC mice exhibit pathological hallmarks of GLD including psychosine accumulation, neuroinflammation, CNS infiltration of macrophages, astrogliosis and demyelination. "
01/25/2014 - "It is pathologically characterized by demyelination of the central and peripheral nervous systems by accumulation of galactosylsphingosine. "
12/01/2013 - "Psychosine accumulation preferentially affects oligodendrocytes, leading to progressive demyelination and infiltration of activated monocytes/macrophages into the CNS. "
01/01/1990 - "These data suggest that neural cells from twitcher mice degenerative with age also in vitro and accumulation of psychosine in twitcher DRG may play an important role for the demyelination in GLD."
01/01/1991 - "Psychosine cytotoxicity was tested as to its effects on rat C6 glioma cells. "
05/01/2002 - "Psychosine potentiated the LPS-induced production of proinflammatory cytokines (IL-1beta, IL-6, and TNF-alpha) in primary rat astrocytes and regulated the cytokine-mediated production of NO in C6 glioma and primary rat astrocyte. "
01/01/1991 - "Psychosine cytotoxicity toward rat C6 glioma cells and the protective effects of phorbol ester and dimethylsulfoxide: implications for therapy in Krabbe disease."
|4.||Gaucher Disease (Gaucher's Disease)
09/01/1982 - "We suggest that the accumulation of the cell-toxic substance psychosine is the basis for the extensive neuronal cell loss in Gaucher disease, which is most striking in Type II disease."
09/14/1982 - "The occurrence of psychosine and other glycolipids in spleen and liver from the three major types of Gaucher's disease."
09/01/1982 - "Accumulation of glucosylceramide and glucosylsphingosine (psychosine) in cerebrum and cerebellum in infantile and juvenile Gaucher disease."
03/01/1998 - "Meanwhile this "psychosine hypothesis" has been extended to Gaucher disease and then to a more general hypothesis encompassing all sphingolipidoses that the "lyso-derivatives" of the primary sphingolipid substrates of the defective enzymes in respective disorders play a key role in their pathogenesis. "
02/15/2014 - "Gaucher disease is caused by the defective activity of the lysosomal enzyme, glucocerebrosidase (GCase; GBA1), resulting in intracellular accumulation of the glycosphingolipids, glucosylceramide and psychosine. "
06/01/2008 - "This study delineates an explicit role for AMPK in psychosine induced inflammation in astrocytes without directly affecting the cell death of oligodendrocytes. "
07/06/2011 - "Although AAV2/5 alone provided supraphysiological levels of GALC activity and reduced psychosine levels in both the brain and spinal cord, it significantly increased CNS inflammation. "
12/01/2011 - "One of a family of devastating lysosomal storage disorders, Krabbe disease is characterized by demyelination, psychosine accumulation, and inflammation. "
06/01/2008 - "Krabbe disease (KD) is an inherited neurological disorder caused by the deficiency of galactocerebrosidase activity resulting in accumulation of psychosine, which leads to energy depletion, loss of oligodendrocytes, induction of gliosis, and inflammation by astrocytes in CNS. "
|1.||Dimethyl Sulfoxide (DMSO)
|7.||Nitric Oxide Synthase Type II (Inducible Nitric Oxide Synthase)
|9.||CDw17 antigen (lactosylceramide)
|1.||Bone Marrow Transplantation (Transplantation, Bone Marrow)
|2.||Enzyme Replacement Therapy