Literature DB >> 10645473

Recent advances in Canavan disease.

R Matalon1, K Michals-Matalon.   

Abstract

More studies are needed to elucidate the pathophysiology of Canavan disease and how the inability to hydrolyze NAA leads to spongy degeneration. The creation of an animal model would be helpful in the understanding of the disease and the formulation of gene therapy.

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Year:  1999        PMID: 10645473

Source DB:  PubMed          Journal:  Adv Pediatr        ISSN: 0065-3101


  10 in total

1.  The impact of structural biology on neurobiology.

Authors:  Ronald E Viola
Journal:  Proc Natl Acad Sci U S A       Date:  2007-01-03       Impact factor: 11.205

2.  Modification of aspartoacylase for potential use in enzyme replacement therapy for the treatment of Canavan disease.

Authors:  Stephen Zano; Radhika Malik; Sylvia Szucs; Reuben Matalon; Ronald E Viola
Journal:  Mol Genet Metab       Date:  2010-10-30       Impact factor: 4.797

3.  Enhanced brain distribution of modified aspartoacylase.

Authors:  Nitesh K Poddar; Stephen Zano; Reka Natarajan; Bryan Yamamoto; Ronald E Viola
Journal:  Mol Genet Metab       Date:  2014-07-12       Impact factor: 4.797

4.  Restricted diffusion in Canavan disease.

Authors:  S G Srikanth; H S Chandrashekar; K Nagarajan; P N Jayakumar
Journal:  Childs Nerv Syst       Date:  2007-01-12       Impact factor: 1.475

5.  A missense mutation (p.G274R) in gene ASPA causes Canavan disease in a Pakistani family.

Authors:  Rashida Hussain; Shakeela Daud; Naseebullah Kakar; Adeel Ahmad; Abdul Hameed Baloch; Abdul Malik Tareen; Muhammad Azam Kakar; Jamil Ahmad
Journal:  Mol Biol Rep       Date:  2012-01-05       Impact factor: 2.316

6.  Characterization of human aspartoacylase: the brain enzyme responsible for Canavan disease.

Authors:  Johanne Le Coq; Hyun-Joo An; Carlito Lebrilla; Ronald E Viola
Journal:  Biochemistry       Date:  2006-05-09       Impact factor: 3.162

7.  Examination of the mechanism of human brain aspartoacylase through the binding of an intermediate analogue.

Authors:  Johanne Le Coq; Alexander Pavlovsky; Radhika Malik; Ruslan Sanishvili; Chengfu Xu; Ronald E Viola
Journal:  Biochemistry       Date:  2008-02-23       Impact factor: 3.162

8.  Mutational analysis of aspartoacylase: implications for Canavan disease.

Authors:  Jeremy R Hershfield; Nagarajan Pattabiraman; Chikkathur N Madhavarao; M A Aryan Namboodiri
Journal:  Brain Res       Date:  2007-03-03       Impact factor: 3.252

Review 9.  Canavan's spongiform leukodystrophy: a clinical anatomy of a genetic metabolic CNS disease.

Authors:  M H Baslow
Journal:  J Mol Neurosci       Date:  2000-10       Impact factor: 2.866

10.  Cell-Based Therapy for Canavan Disease Using Human iPSC-Derived NPCs and OPCs.

Authors:  Lizhao Feng; Jianfei Chao; E Tian; Li Li; Peng Ye; Mi Zhang; Xianwei Chen; Qi Cui; Guihua Sun; Tao Zhou; Gerardo Felix; Yue Qin; Wendong Li; Edward David Meza; Jeremy Klein; Lucy Ghoda; Weidong Hu; Yonglun Luo; Wei Dang; David Hsu; Joseph Gold; Steven A Goldman; Reuben Matalon; Yanhong Shi
Journal:  Adv Sci (Weinh)       Date:  2020-10-29       Impact factor: 16.806

  10 in total

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