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Acid sphingomyelinase-deficient mice mimic the neurovisceral form of human lysosomal storage disease (Niemann-Pick disease)

Article Abstract:

A disease similar to the lethal human sphingomyelinase storage or the Niemann-Pick disease is exhibited by mice with induced deficiency of acid sphingomyelinase (aSMase). Plasma membrane and plasma membrane-derived membranes contain sphingomyelin as the main lipid, and aSMase present in lysosomes of all cells hydrolyzes sphingomyelin to ceramide and phosphocholine. Study of ceramide generation in Niemann-Pick mice which exhibit neuromotor coordination impairment due to accumulation of sphingomyelin may help in somatic gene therapy.

Author: Otterbach, Bernd, Stoffel, Wilhelm
Publisher: Elsevier B.V.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 1995
Observations, Medical genetics, Sphingolipids, Animal mutation

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Identification and disruption of a plant Shaker-like outward channel involved in K+ release into the xylem sap

Article Abstract:

A study showed that the SKOR, a potassium positive ion (K+) channel from Arabidopsis, is the first member of the Shaker family in plants having outwardly rectifying properties and is expressed in root stelar tissues. A reverse genetic approach suggests that SKOR is involved in the K+ release into the xylem sap. It was also found that phytohormone abscisic acid strongly represses SKOR gene expression.

Author: Bouchez, David, Sentenac, Herve, Gaymard, Frederic, Pilot, Guillaume, Lacombe, Benoit, Bruneau, Dominique, Boucherez, Jossia, Michaux-Ferriere, Nicole, Thibaud, Jean-Baptiste
Publisher: Elsevier B.V.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 1998
Plants, Plants (Organisms), Arabidopsis, Potassium (Chemical element), Potassium, Xylem

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