Literature DB >> 116415

The gangliosidoses: comparative features and research applications.

H J Baker, G D Reynolds, S U Walkley, N R Cox, G H Baker.   

Abstract

Ganglioside storage diseases are inherited defects of lysosomal hydrolases that result in intralysosomal accumulation of gangliosides and other complex metabolites. Gangliosidoses occur in man, cats, cattle, dogs and swine. In all species, these diseases are characterized clinically by relentlessly progressive neurological deterioration. Lysosomal hypertrophy with characteristic ultrastructural inclusions occur in neurons, endothelial and other cells. Definitive diagnosis requires biochemical identification of the storage product and enzyme deficiency. Gangliosidoses in animals are important models of human lysosomal diseases and may be a significant complication in the maintenance of certain purebred stocks of domestic animals.

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Year:  1979        PMID： 116415     DOI： 10.1177/030098587901600602

Source DB:  PubMed          Journal:  Vet Pathol        ISSN： 0300-9858            Impact factor:   2.221


  14 in total

1.  Generation and characterization of recombinant feline beta-galactosidase for preclinical enzyme replacement therapy studies in GM1 gangliosidosis.

Authors:  Tatiana I Samoylova; Douglas R Martin; Nancy E Morrison; Misako Hwang; Anna M Cochran; Alexandre M Samoylov; Henry J Baker; Nancy R Cox
Journal:  Metab Brain Dis       Date:  2008-04-18       Impact factor: 3.584

2.  GM2 gangliosidosis in a Japanese spaniel.

Authors:  J F Cummings; P A Wood; S U Walkley; A de Lahunta; M E DeForest
Journal:  Acta Neuropathol       Date:  1985       Impact factor: 17.088

3.  AAV-mediated gene delivery attenuates neuroinflammation in feline Sandhoff disease.

Authors:  Allison M Bradbury; Tiffany A Peterson; Amanda L Gross; Stephen Z Wells; Victoria J McCurdy; Karen G Wolfe; John C Dennis; Brandon L Brunson; Heather Gray-Edwards; Ashley N Randle; Aime K Johnson; Edward E Morrison; Nancy R Cox; Henry J Baker; Miguel Sena-Esteves; Douglas R Martin
Journal:  Neuroscience       Date:  2016-10-26       Impact factor: 3.590

4.  Sustained normalization of neurological disease after intracranial gene therapy in a feline model.

Authors:  Victoria J McCurdy; Aime K Johnson; Heather L Gray-Edwards; Ashley N Randle; Brandon L Brunson; Nancy E Morrison; Nouha Salibi; Jacob A Johnson; Misako Hwang; Ronald J Beyers; Stanley G Leroy; Stacy Maitland; Thomas S Denney; Nancy R Cox; Henry J Baker; Miguel Sena-Esteves; Douglas R Martin
Journal:  Sci Transl Med       Date:  2014-04-09       Impact factor: 17.956

5.  Neurovisceral sphingomyelinosis in a Siamese cat.

Authors:  T Yamagami; M Umeda; S Kamiya; K Sugiyama
Journal:  Acta Neuropathol       Date:  1989       Impact factor: 17.088

6.  Characterization of a new model of GM2-gangliosidosis (Sandhoff's disease) in Korat cats.

Authors:  E A Neuwelt; W G Johnson; N K Blank; M A Pagel; C Maslen-McClure; M J McClure; P M Wu
Journal:  J Clin Invest       Date:  1985-08       Impact factor: 14.808

7.  Molecular consequences of the pathogenic mutation in feline GM1 gangliosidosis.

Authors:  Douglas R Martin; Brigitte A Rigat; Polly Foureman; G S Varadarajan; Misako Hwang; Barbara K Krum; Bruce F Smith; John W Callahan; Don J Mahuran; Henry J Baker
Journal:  Mol Genet Metab       Date:  2008-03-18       Impact factor: 4.797

8.  Phorbol ester receptors in cerebral cortex of cats with GM1 gangliosidosis.

Authors:  G Shanker; H J Baker
Journal:  Neurochem Res       Date:  1991-01       Impact factor: 3.996

9.  Therapeutic response in feline sandhoff disease despite immunity to intracranial gene therapy.

Authors:  Allison M Bradbury; J Nicholas Cochran; Victoria J McCurdy; Aime K Johnson; Brandon L Brunson; Heather Gray-Edwards; Stanley G Leroy; Misako Hwang; Ashley N Randle; Laura S Jackson; Nancy E Morrison; Rena C Baek; Thomas N Seyfried; Seng H Cheng; Nancy R Cox; Henry J Baker; M Begona Cachón-González; Timothy M Cox; Miguel Sena-Esteves; Douglas R Martin
Journal:  Mol Ther       Date:  2013-05-21       Impact factor: 11.454

10.  Sphingomyelin lipidosis in a cat: Golgi studies.

Authors:  S U Walkley; H J Baker
Journal:  Acta Neuropathol       Date:  1984       Impact factor: 17.088
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