Neuronal-visceral GM1 Gangliosidosis in a Dog with Beta-galactosidase Deficiency

Overview

Journal Science

Specialty Science

Date 1976 Oct 22

PMID 824730

Citations 22

Authors

D H Read

D D Harrington

T W Keenana

E J HINSMAN

Affiliations

Soon will be listed here.

Abstract

A 9-month-old dog with a history of progressive motor dysfunction was shown to have a deficiency in brain beta-galactosidase activity. The canine disease, like that of children with GM1 gangliosidosis, is characterized by accumulation of GM1 ganglioside in the brain, liver, and spleen, and membranous cytoplasmic bodies in neurons. The dog's pedigree suggests an autosomal recessive pattern of inheritance.

Citing Articles

Carrier Rate and Mutant Allele Frequency of GM1 Gangliosidosis in Miniature Shiba Inus (Mame Shiba): Population Screening of Breeding Dogs in Japan.

Pervin S, Islam M, Yorisada Y, Sakai A, Masamune S, Yabuki A Animals (Basel). 2022; 12(10).

PMID: 35625088 PMC: 9137666. DOI: 10.3390/ani12101242.

GM1 Gangliosidosis: Mechanisms and Management.

Rha A, Maguire A, Martin D Appl Clin Genet. 2021; 14:209-233.

PMID: 33859490 PMC: 8044076. DOI: 10.2147/TACG.S206076.

Axonopathy and Reduction of Membrane Resistance: Key Features in a New Murine Model of Human G-Gangliosidosis.

Eikelberg D, Lehmbecker A, Brogden G, Tongtako W, Hahn K, Habierski A J Clin Med. 2020; 9(4).

PMID: 32252429 PMC: 7230899. DOI: 10.3390/jcm9041004.

Human knockout cerebral organoids: A model system for testing AAV9-mediated gene therapy for reducing GM1 ganglioside storage in GM1 gangliosidosis.

Latour Y, Yoon R, Thomas S, Grant C, Li C, Sena-Esteves M Mol Genet Metab Rep. 2019; 21:100513.

PMID: 31534909 PMC: 6744524. DOI: 10.1016/j.ymgmr.2019.100513.

In situ detection of GM1 and GM2 gangliosides using immunohistochemical and immunofluorescent techniques for auxiliary diagnosis of canine and feline gangliosidoses.

Kohyama M, Yabuki A, Ochiai K, Nakamoto Y, Uchida K, Hasegawa D BMC Vet Res. 2016; 12:67.

PMID: 27036194 PMC: 4815186. DOI: 10.1186/s12917-016-0691-y.