Literature DB >> 34498299

Ablating the Transporter Sodium-Dependent Dicarboxylate Transporter 3 Prevents Leukodystrophy in Canavan Disease Mice.

Yan Wang1, Vanessa Hull1, Sarah Sternbach2, Brad Popovich3, Travis Burns1, Jennifer McDonough2, Fuzheng Guo1, David Pleasure1.   

Abstract

Canavan disease is caused by ASPA mutations that diminish brain aspartoacylase activity, and it is characterized by excessive brain storage of the aspartoacylase substrate, N-acetyl-l-aspartate (NAA), and by astroglial and intramyelinic vacuolation. Astroglia and the arachnoid mater express sodium-dependent dicarboxylate transporter (NaDC3), encoded by SLC13A3, a sodium-coupled transporter for NAA and other dicarboxylates. Constitutive Slc13a3 deletion in aspartoacylase-deficient Canavan disease mice prevents brain NAA overaccumulation, ataxia, and brain vacuolation. ANN NEUROL 2021;90:845-850.
© 2021 American Neurological Association.

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Year:  2021        PMID： 34498299      PMCID： PMC9549923          DOI： 10.1002/ana.26211

Source DB:  PubMed          Journal:  Ann Neurol        ISSN： 0364-5134            Impact factor:   11.274


  20 in total

1.  ProxTom lymphatic vessel reporter mice reveal Prox1 expression in the adrenal medulla, megakaryocytes, and platelets.

Authors:  Lucy A Truman; Kevin L Bentley; Elenoe C Smith; Stephanie A Massaro; David G Gonzalez; Ann M Haberman; Myriam Hill; Dennis Jones; Wang Min; Diane S Krause; Nancy H Ruddle
Journal:  Am J Pathol       Date:  2012-02-04       Impact factor: 4.307

2.  Lymphatic endothelial cell identity is reversible and its maintenance requires Prox1 activity.

Authors:  Nicole C Johnson; Miriam E Dillard; Peter Baluk; Donald M McDonald; Natasha L Harvey; Sharon L Frase; Guillermo Oliver
Journal:  Genes Dev       Date:  2008-12-01       Impact factor: 11.361

3.  N-Acetylaspartate Synthase Deficiency Corrects the Myelin Phenotype in a Canavan Disease Mouse Model But Does Not Affect Survival Time.

Authors:  Helena Maier; Lihua Wang-Eckhardt; Dieter Hartmann; Volkmar Gieselmann; Matthias Eckhardt
Journal:  J Neurosci       Date:  2015-10-28       Impact factor: 6.167

4.  SLC13A3 variants cause acute reversible leukoencephalopathy and α-ketoglutarate accumulation.

Authors:  Joseph P Dewulf; Elsa Wiame; Imen Dorboz; Monique Elmaleh-Bergès; Apolline Imbard; Dana Dumitriu; Malgorzata Rak; Agnès Bourillon; Raphaël Helaers; Alisha Malla; Florence Renaldo; Odile Boespflug-Tanguy; Marie-Françoise Vincent; Jean-François Benoist; Ron A Wevers; Avner Schlessinger; Emile Van Schaftingen; Marie-Cécile Nassogne; Manuel Schiff
Journal:  Ann Neurol       Date:  2019-01-29       Impact factor: 10.422

5.  Ablating N-acetylaspartate prevents leukodystrophy in a Canavan disease model.

Authors:  Fuzheng Guo; Peter Bannerman; Emily Mills Ko; Laird Miers; Jie Xu; Travis Burns; Shuo Li; Ernest Freeman; Jennifer A McDonough; David Pleasure
Journal:  Ann Neurol       Date:  2015-03-27       Impact factor: 10.422

6.  Long-term follow-up after gene therapy for canavan disease.

Authors:  Paola Leone; David Shera; Scott W J McPhee; Jeremy S Francis; Edwin H Kolodny; Larissa T Bilaniuk; Dah-Jyuu Wang; Mitra Assadi; Olga Goldfarb; H Warren Goldman; Andrew Freese; Deborah Young; Matthew J During; R Jude Samulski; Christopher G Janson
Journal:  Sci Transl Med       Date:  2012-12-19       Impact factor: 17.956

Review 7.  SLC13 family of Na⁺-coupled di- and tri-carboxylate/sulfate transporters.

Authors:  M J Bergeron; B Clémençon; M A Hediger; D Markovich
Journal:  Mol Aspects Med       Date:  2013 Apr-Jun

Review 8.  Spongy degeneration of the central nervous system (van Bogaert and Bertrand type; Canavan's disease). A review.

Authors:  M Adachi; L Schneck; J Cara; B W Volk
Journal:  Hum Pathol       Date:  1973-09       Impact factor: 3.466

9.  The natural history of Canavan disease: 23 new cases and comparison with patients from literature.

Authors:  Annette Bley; Jonas Denecke; Alfried Kohlschütter; Gerhard Schön; Sandra Hischke; Philipp Guder; Tatjana Bierhals; Heather Lau; Maja Hempel; Florian S Eichler
Journal:  Orphanet J Rare Dis       Date:  2021-05-19       Impact factor: 4.123

10.  CNS lymphatic drainage and neuroinflammation are regulated by meningeal lymphatic vasculature.

Authors:  Antoine Louveau; Jasmin Herz; Maria Nordheim Alme; Andrea Francesca Salvador; Michael Q Dong; Kenneth E Viar; S Grace Herod; James Knopp; Joshua C Setliff; Alexander L Lupi; Sandro Da Mesquita; Elizabeth L Frost; Alban Gaultier; Tajie H Harris; Rui Cao; Song Hu; John R Lukens; Igor Smirnov; Christopher C Overall; Guillermo Oliver; Jonathan Kipnis
Journal:  Nat Neurosci       Date:  2018-09-17       Impact factor: 24.884
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