Biochim Biophys Acta. 2002 Dec 19;1573(3):356-62.

Beta1,4-N-acetylgalactosaminyltransferase--GM2/GD2 synthase: a key enzyme to control the synthesis of brain-enriched complex gangliosides.

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Department of Biochemistry II, Nagoya University School of Medicine, 65 Tsurumai, Showa-ku, Nagoya, Japan. koichi@med.nagoya-u.ac.jp

Abstract

Beta1,4-N-acetylgalactosaminyltransferase (GM2/GD2 synthase) is a key enzyme which catalyzes the conversion of GM3, GD3 and lactosylceramide (LacCer) to GM2, GD2 and asialo-GM2 (GA2), respectively. This step is critical for the synthesis of all complex gangliosides enriched in the nervous system of vertebrates. Following the cloning of cDNAs encoding GM2/GD2 synthase by an expression cloning approach, substantial evidence for the roles of complex gangliosides have been obtained. Above all, knock-out mice lacking all complex gangliosides revealed important roles of complex gangliosides in vivo, i.e., in the maintenance and repair of nervous tissues, in the intact differentiation of spermatocytes via the transport of testosterone, and in the regulation of interleukin-2 receptor complex. Molecular mechanisms for these functions of complex gangliosides in vivo remain to be clarified.

PMID:: 12417418; [PubMed - indexed for MEDLINE]

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Cloned beta 1,4 N-acetylgalactosaminyltransferase synthesizes GA2 as well as gangliosides GM2 and GD2. GM3 synthesis has priority over GA2 synthesis for utilization of lactosylceramide substrate in vivo. [J Biol Chem. 1994]
Cloned beta 1,4 N-acetylgalactosaminyltransferase synthesizes GA2 as well as gangliosides GM2 and GD2. GM3 synthesis has priority over GA2 synthesis for utilization of lactosylceramide substrate in vivo.
Lutz MS, Jaskiewicz E, Darling DS, Furukawa K, Young WW Jr. J Biol Chem. 1994 Nov 18; 269(46):29227-31.
Substrate specificity of beta 1,4-N-acetylgalactosaminyltransferase in vitro and in cDNA-transfected cells. GM2/GD2 synthase efficiently generates asialo-GM2 in certain cells. [J Biol Chem. 1995]
Substrate specificity of beta 1,4-N-acetylgalactosaminyltransferase in vitro and in cDNA-transfected cells. GM2/GD2 synthase efficiently generates asialo-GM2 in certain cells.
Yamashiro S, Haraguchi M, Furukawa K, Takamiya K, Yamamoto A, Nagata Y, Lloyd KO, Shiku H, Furukawa K. J Biol Chem. 1995 Mar 17; 270(11):6149-55.
beta 1-4N-acetylgalactosaminyltransferase can synthesize both asialoglycosphingolipid GM2 and glycosphingolipid GM2 in vitro and in vivo: isolation and characterization of a beta 1-4N-acetylgalactosaminyltransferase cDNA clone from rat ascites hepatoma cell line AH7974F. [Biochem J. 1994]
beta 1-4N-acetylgalactosaminyltransferase can synthesize both asialoglycosphingolipid GM2 and glycosphingolipid GM2 in vitro and in vivo: isolation and characterization of a beta 1-4N-acetylgalactosaminyltransferase cDNA clone from rat ascites hepatoma cell line AH7974F.
Hidari JK, Ichikawa S, Furukawa K, Yamasaki M, Hirabayashi Y. Biochem J. 1994 Nov 1; 303 ( Pt 3):957-65.
Review [Cloning of beta 1, 4N-acetylgalactosaminyltransferase cDNAs and its application to the function analysis of carbohydrates]. [Seikagaku. 1993]
Review [Cloning of beta 1, 4N-acetylgalactosaminyltransferase cDNAs and its application to the function analysis of carbohydrates].
Furukawa K. Seikagaku. 1993 Jul; 65(7):566-9.
Review [Function analysis of complex gangliosides by gangliosides-lacking mutant mice]. [Tanpakushitsu Kakusan Koso. 1998]
Review [Function analysis of complex gangliosides by gangliosides-lacking mutant mice].
Furukawa K, Takamiya K, Fukumoto S, Zhao J, Okada M, Furukawa K, Miyazaki H. Tanpakushitsu Kakusan Koso. 1998 Dec; 43(16 Suppl):2295-302.

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