Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0162473 (Frey)
 2,599 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Gangliosides are a family of sialic acid-containing glycosphingolipids that are highly enriched in the mammalian nervous system. In particular, b- and c-series gangliosides, all of which contain alpha-2,8 sialic acids, have been considered to play important roles in adhesion, toxin-binding, neurite extension, cell growth and apoptosis. However, the neurobiological functions of these series of gangliosides remain largely unknown. To clarify the function of b- and c-series gangliosides in pain sensation in vivo, we generated mice in whom the gene for the alpha-2,8-sialyltransferase (GD3 synthase), which is responsible for the generation of all b-series gangliosides as well as c-series gangliosides, was disrupted. Compared to the wild-type mice, the mutant mice exhibited increased sensory responses to thermal and mechanical stimuli as measured by a hot plate test and von Frey test. In contrast, the mutant mice showed decreased responses during the late phase of the formalin test. Paw edema and Fos expression in the spinal cord after formalin injection were significantly decreased in the mutant mice compared to the wild-type mice. No significant differences in the conduction velocity of the sciatic nerve, and no apparent morphologic differences in the spinal cord and the sciatic nerve were detected between the wild-type and the mutant mice. These results suggested that b- and c-series gangliosides are critical in the development and/or maintenance of the sensory nervous system responsible for the transmission of acute pain sensation and pain modulation. Moreover, they play an important role in the development of hyperalgesia induced by inflammation.
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PMID:GD3 synthase gene knockout mice exhibit thermal hyperalgesia and mechanical allodynia but decreased response to formalin-induced prolonged noxious stimulation. 1615 May 45

Sialic acid-containing glycosphingolipids (gangliosides) have been believed to play a role in the regulation and protection of nervous tissues. To clarify their function in the nervous system in vivo, double knockout (DKO) mice of GM2/GD2 synthase and GD3 synthase genes were generated and abnormal behaviors were analyzed. Mutant mice exhibited reduced weight and a round shape of the whole brain that progressively emerged with aging, and displayed motor dysfunction in the footprint, traction, open-field, and 24h locomotion activity tests. Sensory functions were also reduced in the von Frey and hot plate tests and greatly reduced in the acoustic startle response test. For emotional behavior, fear response was clearly decreased. Numerous neuronal dysfunctions were found even in younger mutant mice examined at 10-23 weeks after birth, which were exacerbated with aging. These results suggest that a lack of gangliosides other than GM3 induces severe neuronal degeneration in the early stage of life, and that the expression of complex gangliosides is essential to maintain the integrity of the nervous system throughout life.
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PMID:Reduced motor and sensory functions and emotional response in GM3-only mice: emergence from early stage of life and exacerbation with aging. 1901 84