Literature DB >> 22078456

Developing treatment options for metachromatic leukodystrophy.

Spyros P Batzios1, Dimitrios I Zafeiriou.   

Abstract

Metachromatic leukodystrophy (MLD) represents a devastating lysosomal storage disease characterized by intralysosomal accumulation of the sphingolipid sulfatide in various tissues. Three types of the disease are currently distinguished: the late-infantile, which is the most commonly observed, the juvenile and the adult type. Demyelination represents the main histopathological feature of the disorder, leading to neurological impairment with no curative treatment currently available. Nevertheless, the increased scientific interest on the disease has led to the experimental use of innovative therapeutic approaches in animal models, aiming to provide an effective therapeutic regimen for human patients, as well. This paper provides an overview of developing treatment options among patients with MLD. Apart from hematopoietic stem cell transplantation, already in use for decades, other recent data discussed includes umbilical cord blood and stem cell transplantation, enzyme replacement therapy, gene therapy and autologous hematopoietic transplantation of genetically modified stem cells. Gene therapy with oligodedroglial, neural progenitor, embryonic and microencapsulated recombinant cells represents add-on treatment options still on experimental level.
Copyright © 2011 Elsevier Inc. All rights reserved.

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Year:  2011        PMID: 22078456     DOI: 10.1016/j.ymgme.2011.10.002

Source DB:  PubMed          Journal:  Mol Genet Metab        ISSN: 1096-7192            Impact factor:   4.797


  14 in total

1.  Direct tandem mass spectrometric profiling of sulfatides in dry urinary samples for screening of metachromatic leukodystrophy.

Authors:  Ladislav Kuchař; Befekadu Asfaw; Helena Poupětová; Jitka Honzíková; František Tureček; Jana Ledvinová
Journal:  Clin Chim Acta       Date:  2013-07-06       Impact factor: 3.786

Review 2.  Gene therapy for metachromatic leukodystrophy.

Authors:  Jonathan B Rosenberg; Stephen M Kaminsky; Patrick Aubourg; Ronald G Crystal; Dolan Sondhi
Journal:  J Neurosci Res       Date:  2016-11       Impact factor: 4.164

3.  Comparative efficacy and safety of multiple routes of direct CNS administration of adeno-associated virus gene transfer vector serotype rh.10 expressing the human arylsulfatase A cDNA to nonhuman primates.

Authors:  Jonathan B Rosenberg; Dolan Sondhi; David G Rubin; Sébastien Monette; Alvin Chen; Sara Cram; Bishnu P De; Stephen M Kaminsky; Caroline Sevin; Patrick Aubourg; Ronald G Crystal
Journal:  Hum Gene Ther Clin Dev       Date:  2014-08-21       Impact factor: 5.032

4.  Rare Diseases in Glycosphingolipid Metabolism.

Authors:  Hongwen Zhou; Zhoulu Wu; Yiwen Wang; Qinyi Wu; Moran Hu; Shuai Ma; Min Zhou; Yan Sun; Baowen Yu; Jingya Ye; Wanzi Jiang; Zhenzhen Fu; Yingyun Gong
Journal:  Adv Exp Med Biol       Date:  2022       Impact factor: 2.622

5.  Safety of Direct Intraparenchymal AAVrh.10-Mediated Central Nervous System Gene Therapy for Metachromatic Leukodystrophy.

Authors:  Jonathan B Rosenberg; Alvin Chen; Bishnu P De; Jonathan P Dyke; Douglas J Ballon; Sebastien Monette; Rodolfo J Ricart Arbona; Stephen M Kaminsky; Ronald G Crystal; Dolan Sondhi
Journal:  Hum Gene Ther       Date:  2021-03-30       Impact factor: 4.793

6.  Volumetric MRI data correlate to disease severity in metachromatic leukodystrophy.

Authors:  Jan-Mendelt Tillema; Marloes Gm Derks; Petra J W Pouwels; Pim de Graaf; Diane F van Rappard; Frederik Barkhof; Marjan E Steenweg; Marjo S van der Knaap; Nicole I Wolf
Journal:  Ann Clin Transl Neurol       Date:  2015-08-24       Impact factor: 4.511

7.  Long-term outcomes after allogeneic hematopoietic stem cell transplantation for metachromatic leukodystrophy: the largest single-institution cohort report.

Authors:  Alexander A Boucher; Weston Miller; Ryan Shanley; Richard Ziegler; Troy Lund; Gerald Raymond; Paul J Orchard
Journal:  Orphanet J Rare Dis       Date:  2015-08-07       Impact factor: 4.123

Review 8.  Developing therapeutic approaches for metachromatic leukodystrophy.

Authors:  Shilpa A Patil; Gustavo H B Maegawa
Journal:  Drug Des Devel Ther       Date:  2013-08-08       Impact factor: 4.162

9.  Language and cognition in children with metachromatic leukodystrophy: onset and natural course in a nationwide cohort.

Authors:  Christiane Kehrer; Samuel Groeschel; Birgit Kustermann-Kuhn; Friederike Bürger; Wolfgang Köhler; Alfried Kohlschütter; Annette Bley; Robert Steinfeld; Volkmar Gieselmann; Ingeborg Krägeloh-Mann
Journal:  Orphanet J Rare Dis       Date:  2014-02-05       Impact factor: 4.123

10.  Four novel ARSA gene mutations with pathogenic impacts on metachromatic leukodystrophy: a bioinformatics approach to predict pathogenic mutations.

Authors:  Masoumeh Dehghan Manshadi; Behnam Kamalidehghan; Omid Aryani; Elham Khalili; Sepideh Dadgar; Mahdi Tondar; Fatemeh Ahmadipour; Goh Yong Meng; Massoud Houshmand
Journal:  Ther Clin Risk Manag       Date:  2017-06-16       Impact factor: 2.423

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