Literature DB >> 15085187

Blood to brain to the rescue.

Richard L Proia1, Yun-Ping Wu.   

Abstract

Neurodegeneration occurs in the majority of the more than 40 known lysosomal storage diseases. Since the nervous system in these disorders can be globally affected, effective treatment would require persistent widespread correction. Biffi et al. show such correction is possible in a mouse model of metachromatic leukodystrophy by the transplantation of hematopoietic cells genetically modified to overexpress the missing lysosomal enzyme. The results reveal a nervous system damage-response pathway that can be harnessed to provide therapy to the nervous system in these serious disorders.

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Year:  2004        PMID: 15085187      PMCID: PMC385411          DOI: 10.1172/JCI21476

Source DB:  PubMed          Journal:  J Clin Invest        ISSN: 0021-9738            Impact factor:   14.808


  14 in total

1.  Turning blood into brain: cells bearing neuronal antigens generated in vivo from bone marrow.

Authors:  E Mezey; K J Chandross; G Harta; R A Maki; S R McKercher
Journal:  Science       Date:  2000-12-01       Impact factor: 47.728

2.  Targeting gene-modified hematopoietic cells to the central nervous system: use of green fluorescent protein uncovers microglial engraftment.

Authors:  J Priller; A Flügel; T Wehner; M Boentert; C A Haas; M Prinz; F Fernández-Klett; K Prass; I Bechmann; B A de Boer; M Frotscher; G W Kreutzberg; D A Persons; U Dirnagl
Journal:  Nat Med       Date:  2001-12       Impact factor: 53.440

3.  From marrow to brain: expression of neuronal phenotypes in adult mice.

Authors:  T R Brazelton; F M Rossi; G I Keshet; H M Blau
Journal:  Science       Date:  2000-12-01       Impact factor: 47.728

4.  Functional correction of established central nervous system deficits in an animal model of lysosomal storage disease with feline immunodeficiency virus-based vectors.

Authors:  Andrew I Brooks; Colleen S Stein; Stephanie M Hughes; Jason Heth; Paul M McCray; Sybille L Sauter; Julie C Johnston; Deborah A Cory-Slechta; Howard J Federoff; Beverly L Davidson
Journal:  Proc Natl Acad Sci U S A       Date:  2002-04-16       Impact factor: 11.205

5.  Microglial activation precedes acute neurodegeneration in Sandhoff disease and is suppressed by bone marrow transplantation.

Authors:  R Wada; C J Tifft; R L Proia
Journal:  Proc Natl Acad Sci U S A       Date:  2000-09-26       Impact factor: 11.205

6.  Enzyme-replacement therapy in mucopolysaccharidosis I.

Authors:  E D Kakkis; J Muenzer; G E Tiller; L Waber; J Belmont; M Passage; B Izykowski; J Phillips; R Doroshow; I Walot; R Hoft; E F Neufeld
Journal:  N Engl J Med       Date:  2001-01-18       Impact factor: 91.245

7.  Accelerated transport and maturation of lysosomal alpha-galactosidase A in Fabry lymphoblasts by an enzyme inhibitor.

Authors:  J Q Fan; S Ishii; N Asano; Y Suzuki
Journal:  Nat Med       Date:  1999-01       Impact factor: 53.440

8.  Neural progenitor cell engraftment corrects lysosomal storage throughout the MPS VII mouse brain.

Authors:  E Y Snyder; R M Taylor; J H Wolfe
Journal:  Nature       Date:  1995-03-23       Impact factor: 49.962

Review 9.  Enzyme replacement therapy: conception, chaos and culmination.

Authors:  Roscoe O Brady
Journal:  Philos Trans R Soc Lond B Biol Sci       Date:  2003-05-29       Impact factor: 6.237

10.  Correction of metachromatic leukodystrophy in the mouse model by transplantation of genetically modified hematopoietic stem cells.

Authors:  Alessandra Biffi; Michele De Palma; Angelo Quattrini; Ubaldo Del Carro; Stefano Amadio; Ilaria Visigalli; Maria Sessa; Stefania Fasano; Riccardo Brambilla; Sergio Marchesini; Claudio Bordignon; Luigi Naldini
Journal:  J Clin Invest       Date:  2004-04       Impact factor: 14.808

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  13 in total

Review 1.  Pathophysiology of neuropathic lysosomal storage disorders.

Authors:  Cinzia Maria Bellettato; Maurizio Scarpa
Journal:  J Inherit Metab Dis       Date:  2010-04-29       Impact factor: 4.982

2.  Cln3-mutations underlying juvenile neuronal ceroid lipofuscinosis cause significantly reduced levels of Palmitoyl-protein thioesterases-1 (Ppt1)-protein and Ppt1-enzyme activity in the lysosome.

Authors:  Abhilash P Appu; Maria B Bagh; Tamal Sadhukhan; Avisek Mondal; Sydney Casey; Anil B Mukherjee
Journal:  J Inherit Metab Dis       Date:  2019-05-14       Impact factor: 4.982

3.  Disruption of adaptive energy metabolism and elevated ribosomal p-S6K1 levels contribute to INCL pathogenesis: partial rescue by resveratrol.

Authors:  Hui Wei; Zhongjian Zhang; Arjun Saha; Shiyong Peng; Goutam Chandra; Zenaide Quezado; Anil B Mukherjee
Journal:  Hum Mol Genet       Date:  2010-12-28       Impact factor: 6.150

4.  Substrate reduction therapy of glycosphingolipid storage disorders.

Authors:  Johannes M F G Aerts; Carla E M Hollak; Rolf G Boot; Johanna E M Groener; Mario Maas
Journal:  J Inherit Metab Dis       Date:  2006 Apr-Jun       Impact factor: 4.982

5.  Cln1 gene disruption in mice reveals a common pathogenic link between two of the most lethal childhood neurodegenerative lysosomal storage disorders.

Authors:  Goutam Chandra; Maria B Bagh; Shiyong Peng; Arjun Saha; Chinmoy Sarkar; Matthew Moralle; Zhongjian Zhang; Anil B Mukherjee
Journal:  Hum Mol Genet       Date:  2015-07-09       Impact factor: 6.150

6.  Evaluation of neurodegeneration in a mouse model of infantile batten disease by magnetic resonance imaging and magnetic resonance spectroscopy.

Authors:  Jeeva Munasinghe; Zhongjian Zhang; Eryan Kong; Alison Heffer; Anil B Mukherjee
Journal:  Neurodegener Dis       Date:  2012-02-09       Impact factor: 2.977

7.  Neural stem cell transplantation benefits a monogenic neurometabolic disorder during the symptomatic phase of disease.

Authors:  Mylvaganam Jeyakumar; Jean-Pyo Lee; Nicola R Sibson; John P Lowe; Daniel J Stuckey; Katie Tester; Gerald Fu; Robin Newlin; David A Smith; Evan Y Snyder; Frances M Platt
Journal:  Stem Cells       Date:  2009-09       Impact factor: 6.277

8.  Ppt1-deficiency dysregulates lysosomal Ca++ homeostasis contributing to pathogenesis in a mouse model of CLN1 disease.

Authors:  Avisek Mondal; Abhilash P Appu; Tamal Sadhukhan; Maria B Bagh; Rafael M Previde; Sriparna Sadhukhan; Stanko Stojilkovic; Aiyi Liu; Anil B Mukherjee
Journal:  J Inherit Metab Dis       Date:  2022-03-17       Impact factor: 4.750

9.  Cln1-mutations suppress Rab7-RILP interaction and impair autophagy contributing to neuropathology in a mouse model of infantile neuronal ceroid lipofuscinosis.

Authors:  Chinmoy Sarkar; Tamal Sadhukhan; Maria B Bagh; Abhilash P Appu; Goutam Chandra; Avisek Mondal; Arjun Saha; Anil B Mukherjee
Journal:  J Inherit Metab Dis       Date:  2020-04-27       Impact factor: 4.750

Review 10.  The role of microglia in human disease: therapeutic tool or target?

Authors:  Nathalie Cartier; Coral-Ann Lewis; Regan Zhang; Fabio M V Rossi
Journal:  Acta Neuropathol       Date:  2014-08-09       Impact factor: 17.088

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