Biosynthesis of the major brain gangliosides GD1a and GT1b
- Autores
- Sturgill, Elizabeth R.; Aoki, Kazuhiro; Lopez, Pablo; Colacurcio, Daniel; Vajn, Katarina; Lorenzini, Ileana; Majic, Senka; Yang, Won Ho; Heffer, Marija; Tiemeyer, Michael; Marth, Jamey D.; Schnaar, Ronald L.
- Año de publicación
- 2012
- Idioma
- inglés
- Tipo de recurso
- artículo
- Estado
- versión publicada
- Descripción
- Gangliosides-sialylated glycosphingolipids-are the major glycoconjugates of nerve cells. The same four structures-GM1, GD1a, GD1b and GT1b-comprise the great majority of gangliosides in mammalian brains. They share a common tetrasaccharide core (Gal1-3GalNAc1-4Gal1-4Glc1-1′Cer) with one or two sialic acids on the internal galactose and zero (GM1 and GD1b) or one (GD1a and GT1b) 2-3-linked sialic acid on the terminal galactose. Whereas the genes responsible for the sialylation of the internal galactose are known, those responsible for terminal sialylation have not been established in vivo. We report that St3gal2 and St3gal3 are responsible for nearly all the terminal sialylation of brain gangliosides in the mouse. When brain ganglioside expression was analyzed in adult St3gal1-, St3gal2-, St3gal3-and St3gal4-null mice, only St3gal2-null mice differed significantly from wild type, expressing half the normal amount of GD1a and GT1b. St3gal1/2-double-null mice were no different than St3gal2-single-null mice; however, St3gal2/3-double-null mice were >95 depleted in gangliosides GD1a and GT1b. Total ganglioside expression (lipid-bound sialic acid) in the brains of St3gal2/3-double-null mice was equivalent to that in wild-type mice, whereas total protein sialylation was reduced by half. St3gal2/3-double-null mice were small, weak and short lived. They were half the weight of wild-type mice at weaning and displayed early hindlimb dysreflexia. We conclude that the St3gal2 and St3gal3 gene products (ST3Gal-II and ST3Gal-III sialyltransferases) are largely responsible for ganglioside terminal 2-3 sialylation in the brain, synthesizing the major brain gangliosides GD1a and GT1b. © 2012 The Author.
Fil: Sturgill, Elizabeth R.. Johns Hopkins School Of Medicine; Estados Unidos. University Johns Hopkins; Estados Unidos
Fil: Aoki, Kazuhiro. University of Georgia; Estados Unidos
Fil: Lopez, Pablo. University Johns Hopkins; Estados Unidos
Fil: Colacurcio, Daniel. University Johns Hopkins; Estados Unidos
Fil: Vajn, Katarina. University Johns Hopkins; Estados Unidos
Fil: Lorenzini, Ileana. University Johns Hopkins; Estados Unidos
Fil: Majic, Senka. University Johns Hopkins; Estados Unidos
Fil: Yang, Won Ho. University of California; Estados Unidos
Fil: Heffer, Marija. J. J. Strossmayer University; Croacia
Fil: Tiemeyer, Michael. University of Georgia; Estados Unidos
Fil: Marth, Jamey D.. University of California; Estados Unidos
Fil: Schnaar, Ronald L.. University Johns Hopkins; Estados Unidos - Materia
-
BRAIN
GANGLIOSIDE
MYELIN
SIALIC ACID
SIALYLTRANSFERASE - Nivel de accesibilidad
- acceso abierto
- Condiciones de uso
- https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
- Repositorio
- Institución
- Consejo Nacional de Investigaciones Científicas y Técnicas
- OAI Identificador
- oai:ri.conicet.gov.ar:11336/197563
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network_name_str |
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spelling |
Biosynthesis of the major brain gangliosides GD1a and GT1bSturgill, Elizabeth R.Aoki, KazuhiroLopez, PabloColacurcio, DanielVajn, KatarinaLorenzini, IleanaMajic, SenkaYang, Won HoHeffer, MarijaTiemeyer, MichaelMarth, Jamey D.Schnaar, Ronald L.BRAINGANGLIOSIDEMYELINSIALIC ACIDSIALYLTRANSFERASEhttps://purl.org/becyt/ford/1.6https://purl.org/becyt/ford/1Gangliosides-sialylated glycosphingolipids-are the major glycoconjugates of nerve cells. The same four structures-GM1, GD1a, GD1b and GT1b-comprise the great majority of gangliosides in mammalian brains. They share a common tetrasaccharide core (Gal1-3GalNAc1-4Gal1-4Glc1-1′Cer) with one or two sialic acids on the internal galactose and zero (GM1 and GD1b) or one (GD1a and GT1b) 2-3-linked sialic acid on the terminal galactose. Whereas the genes responsible for the sialylation of the internal galactose are known, those responsible for terminal sialylation have not been established in vivo. We report that St3gal2 and St3gal3 are responsible for nearly all the terminal sialylation of brain gangliosides in the mouse. When brain ganglioside expression was analyzed in adult St3gal1-, St3gal2-, St3gal3-and St3gal4-null mice, only St3gal2-null mice differed significantly from wild type, expressing half the normal amount of GD1a and GT1b. St3gal1/2-double-null mice were no different than St3gal2-single-null mice; however, St3gal2/3-double-null mice were >95 depleted in gangliosides GD1a and GT1b. Total ganglioside expression (lipid-bound sialic acid) in the brains of St3gal2/3-double-null mice was equivalent to that in wild-type mice, whereas total protein sialylation was reduced by half. St3gal2/3-double-null mice were small, weak and short lived. They were half the weight of wild-type mice at weaning and displayed early hindlimb dysreflexia. We conclude that the St3gal2 and St3gal3 gene products (ST3Gal-II and ST3Gal-III sialyltransferases) are largely responsible for ganglioside terminal 2-3 sialylation in the brain, synthesizing the major brain gangliosides GD1a and GT1b. © 2012 The Author.Fil: Sturgill, Elizabeth R.. Johns Hopkins School Of Medicine; Estados Unidos. University Johns Hopkins; Estados UnidosFil: Aoki, Kazuhiro. University of Georgia; Estados UnidosFil: Lopez, Pablo. University Johns Hopkins; Estados UnidosFil: Colacurcio, Daniel. University Johns Hopkins; Estados UnidosFil: Vajn, Katarina. University Johns Hopkins; Estados UnidosFil: Lorenzini, Ileana. University Johns Hopkins; Estados UnidosFil: Majic, Senka. University Johns Hopkins; Estados UnidosFil: Yang, Won Ho. University of California; Estados UnidosFil: Heffer, Marija. J. J. Strossmayer University; CroaciaFil: Tiemeyer, Michael. University of Georgia; Estados UnidosFil: Marth, Jamey D.. University of California; Estados UnidosFil: Schnaar, Ronald L.. University Johns Hopkins; Estados UnidosOxford Univ Press Inc2012-10info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/197563Sturgill, Elizabeth R.; Aoki, Kazuhiro; Lopez, Pablo; Colacurcio, Daniel; Vajn, Katarina; et al.; Biosynthesis of the major brain gangliosides GD1a and GT1b; Oxford Univ Press Inc; Glycobiology; 22; 10; 10-2012; 1289-13010959-66581460-2423CONICET DigitalCONICETenginfo:eu-repo/semantics/altIdentifier/doi/10.1093/glycob/cws103info:eu-repo/semantics/altIdentifier/url/https://academic.oup.com/glycob/article/22/10/1289/1988226info:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by-nc-sa/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-09-03T10:10:47Zoai:ri.conicet.gov.ar:11336/197563instacron:CONICETInstitucionalhttp://ri.conicet.gov.ar/Organismo científico-tecnológicoNo correspondehttp://ri.conicet.gov.ar/oai/requestdasensio@conicet.gov.ar; lcarlino@conicet.gov.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:34982025-09-03 10:10:47.862CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse |
dc.title.none.fl_str_mv |
Biosynthesis of the major brain gangliosides GD1a and GT1b |
title |
Biosynthesis of the major brain gangliosides GD1a and GT1b |
spellingShingle |
Biosynthesis of the major brain gangliosides GD1a and GT1b Sturgill, Elizabeth R. BRAIN GANGLIOSIDE MYELIN SIALIC ACID SIALYLTRANSFERASE |
title_short |
Biosynthesis of the major brain gangliosides GD1a and GT1b |
title_full |
Biosynthesis of the major brain gangliosides GD1a and GT1b |
title_fullStr |
Biosynthesis of the major brain gangliosides GD1a and GT1b |
title_full_unstemmed |
Biosynthesis of the major brain gangliosides GD1a and GT1b |
title_sort |
Biosynthesis of the major brain gangliosides GD1a and GT1b |
dc.creator.none.fl_str_mv |
Sturgill, Elizabeth R. Aoki, Kazuhiro Lopez, Pablo Colacurcio, Daniel Vajn, Katarina Lorenzini, Ileana Majic, Senka Yang, Won Ho Heffer, Marija Tiemeyer, Michael Marth, Jamey D. Schnaar, Ronald L. |
author |
Sturgill, Elizabeth R. |
author_facet |
Sturgill, Elizabeth R. Aoki, Kazuhiro Lopez, Pablo Colacurcio, Daniel Vajn, Katarina Lorenzini, Ileana Majic, Senka Yang, Won Ho Heffer, Marija Tiemeyer, Michael Marth, Jamey D. Schnaar, Ronald L. |
author_role |
author |
author2 |
Aoki, Kazuhiro Lopez, Pablo Colacurcio, Daniel Vajn, Katarina Lorenzini, Ileana Majic, Senka Yang, Won Ho Heffer, Marija Tiemeyer, Michael Marth, Jamey D. Schnaar, Ronald L. |
author2_role |
author author author author author author author author author author author |
dc.subject.none.fl_str_mv |
BRAIN GANGLIOSIDE MYELIN SIALIC ACID SIALYLTRANSFERASE |
topic |
BRAIN GANGLIOSIDE MYELIN SIALIC ACID SIALYLTRANSFERASE |
purl_subject.fl_str_mv |
https://purl.org/becyt/ford/1.6 https://purl.org/becyt/ford/1 |
dc.description.none.fl_txt_mv |
Gangliosides-sialylated glycosphingolipids-are the major glycoconjugates of nerve cells. The same four structures-GM1, GD1a, GD1b and GT1b-comprise the great majority of gangliosides in mammalian brains. They share a common tetrasaccharide core (Gal1-3GalNAc1-4Gal1-4Glc1-1′Cer) with one or two sialic acids on the internal galactose and zero (GM1 and GD1b) or one (GD1a and GT1b) 2-3-linked sialic acid on the terminal galactose. Whereas the genes responsible for the sialylation of the internal galactose are known, those responsible for terminal sialylation have not been established in vivo. We report that St3gal2 and St3gal3 are responsible for nearly all the terminal sialylation of brain gangliosides in the mouse. When brain ganglioside expression was analyzed in adult St3gal1-, St3gal2-, St3gal3-and St3gal4-null mice, only St3gal2-null mice differed significantly from wild type, expressing half the normal amount of GD1a and GT1b. St3gal1/2-double-null mice were no different than St3gal2-single-null mice; however, St3gal2/3-double-null mice were >95 depleted in gangliosides GD1a and GT1b. Total ganglioside expression (lipid-bound sialic acid) in the brains of St3gal2/3-double-null mice was equivalent to that in wild-type mice, whereas total protein sialylation was reduced by half. St3gal2/3-double-null mice were small, weak and short lived. They were half the weight of wild-type mice at weaning and displayed early hindlimb dysreflexia. We conclude that the St3gal2 and St3gal3 gene products (ST3Gal-II and ST3Gal-III sialyltransferases) are largely responsible for ganglioside terminal 2-3 sialylation in the brain, synthesizing the major brain gangliosides GD1a and GT1b. © 2012 The Author. Fil: Sturgill, Elizabeth R.. Johns Hopkins School Of Medicine; Estados Unidos. University Johns Hopkins; Estados Unidos Fil: Aoki, Kazuhiro. University of Georgia; Estados Unidos Fil: Lopez, Pablo. University Johns Hopkins; Estados Unidos Fil: Colacurcio, Daniel. University Johns Hopkins; Estados Unidos Fil: Vajn, Katarina. University Johns Hopkins; Estados Unidos Fil: Lorenzini, Ileana. University Johns Hopkins; Estados Unidos Fil: Majic, Senka. University Johns Hopkins; Estados Unidos Fil: Yang, Won Ho. University of California; Estados Unidos Fil: Heffer, Marija. J. J. Strossmayer University; Croacia Fil: Tiemeyer, Michael. University of Georgia; Estados Unidos Fil: Marth, Jamey D.. University of California; Estados Unidos Fil: Schnaar, Ronald L.. University Johns Hopkins; Estados Unidos |
description |
Gangliosides-sialylated glycosphingolipids-are the major glycoconjugates of nerve cells. The same four structures-GM1, GD1a, GD1b and GT1b-comprise the great majority of gangliosides in mammalian brains. They share a common tetrasaccharide core (Gal1-3GalNAc1-4Gal1-4Glc1-1′Cer) with one or two sialic acids on the internal galactose and zero (GM1 and GD1b) or one (GD1a and GT1b) 2-3-linked sialic acid on the terminal galactose. Whereas the genes responsible for the sialylation of the internal galactose are known, those responsible for terminal sialylation have not been established in vivo. We report that St3gal2 and St3gal3 are responsible for nearly all the terminal sialylation of brain gangliosides in the mouse. When brain ganglioside expression was analyzed in adult St3gal1-, St3gal2-, St3gal3-and St3gal4-null mice, only St3gal2-null mice differed significantly from wild type, expressing half the normal amount of GD1a and GT1b. St3gal1/2-double-null mice were no different than St3gal2-single-null mice; however, St3gal2/3-double-null mice were >95 depleted in gangliosides GD1a and GT1b. Total ganglioside expression (lipid-bound sialic acid) in the brains of St3gal2/3-double-null mice was equivalent to that in wild-type mice, whereas total protein sialylation was reduced by half. St3gal2/3-double-null mice were small, weak and short lived. They were half the weight of wild-type mice at weaning and displayed early hindlimb dysreflexia. We conclude that the St3gal2 and St3gal3 gene products (ST3Gal-II and ST3Gal-III sialyltransferases) are largely responsible for ganglioside terminal 2-3 sialylation in the brain, synthesizing the major brain gangliosides GD1a and GT1b. © 2012 The Author. |
publishDate |
2012 |
dc.date.none.fl_str_mv |
2012-10 |
dc.type.none.fl_str_mv |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion http://purl.org/coar/resource_type/c_6501 info:ar-repo/semantics/articulo |
format |
article |
status_str |
publishedVersion |
dc.identifier.none.fl_str_mv |
http://hdl.handle.net/11336/197563 Sturgill, Elizabeth R.; Aoki, Kazuhiro; Lopez, Pablo; Colacurcio, Daniel; Vajn, Katarina; et al.; Biosynthesis of the major brain gangliosides GD1a and GT1b; Oxford Univ Press Inc; Glycobiology; 22; 10; 10-2012; 1289-1301 0959-6658 1460-2423 CONICET Digital CONICET |
url |
http://hdl.handle.net/11336/197563 |
identifier_str_mv |
Sturgill, Elizabeth R.; Aoki, Kazuhiro; Lopez, Pablo; Colacurcio, Daniel; Vajn, Katarina; et al.; Biosynthesis of the major brain gangliosides GD1a and GT1b; Oxford Univ Press Inc; Glycobiology; 22; 10; 10-2012; 1289-1301 0959-6658 1460-2423 CONICET Digital CONICET |
dc.language.none.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
info:eu-repo/semantics/altIdentifier/doi/10.1093/glycob/cws103 info:eu-repo/semantics/altIdentifier/url/https://academic.oup.com/glycob/article/22/10/1289/1988226 |
dc.rights.none.fl_str_mv |
info:eu-repo/semantics/openAccess https://creativecommons.org/licenses/by-nc-sa/2.5/ar/ |
eu_rights_str_mv |
openAccess |
rights_invalid_str_mv |
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/ |
dc.format.none.fl_str_mv |
application/pdf application/pdf |
dc.publisher.none.fl_str_mv |
Oxford Univ Press Inc |
publisher.none.fl_str_mv |
Oxford Univ Press Inc |
dc.source.none.fl_str_mv |
reponame:CONICET Digital (CONICET) instname:Consejo Nacional de Investigaciones Científicas y Técnicas |
reponame_str |
CONICET Digital (CONICET) |
collection |
CONICET Digital (CONICET) |
instname_str |
Consejo Nacional de Investigaciones Científicas y Técnicas |
repository.name.fl_str_mv |
CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicas |
repository.mail.fl_str_mv |
dasensio@conicet.gov.ar; lcarlino@conicet.gov.ar |
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1842270132454293504 |
score |
13.13397 |