Initiation and growth of ectopic neurites and meganeurites during postnatal cortical development in ganglioside storage disease

Brain Research. Developmental Brain Research
Steven U WalkleyM C Rattazzi

Abstract

The incidence of cortical pyramidal neurons displaying meganeurites or enlarged axon hillocks with ectopic spines and neurites was evaluated developmentally using feline models of GM1 and GM2 gangliosidosis. Results of these studies demonstrated that the onset of ectopic neurite growth occurred after the elaboration of dendrites on cortical pyramidal neurons, and that the time of onset of this renewed dendritogenesis was similar in the two diseases. Initiation and growth of ectopic neurites also correlated in a general way with onset and progression of clinical deterioration in both diseases. In GM1 gangliosidosis there was a greater tendency toward formation of meganeurites, whereas in cats with GM2 gangliosidosis the growth of ectopic axon hillock neurites without meganeurites predominated. At end-stage disease in GM2 gangliosidosis, nearly 90% of pyramidal cells displayed some degree of axon hillock neurite growth as opposed to less than half this number for GM1 gangliosidosis cats at the same age. These data are consistent with the hypothesis that there are two separate driving forces behind these somadendritic abnormalities of pyramidal neurons in the gangliosidoses. Excessive intraneuronal accumulation of storage vacuoles...Continue Reading

References

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Citations

May 24, 2013·Journal of Inherited Metabolic Disease·Mark WalterfangDennis Velakoulis
Jun 28, 2002·Brain Research. Brain Research Reviews·John C FialaKristen M Harris
May 22, 2013·Molecular Therapy : the Journal of the American Society of Gene Therapy·Allison M BradburyDouglas R Martin
Dec 15, 1991·Proceedings of the National Academy of Sciences of the United States of America·L A GoodmanS U Walkley
Jul 21, 1998·Annals of the New York Academy of Sciences·S U WalkleyM Zervas
Nov 1, 1995·Neurochemical Research·S U WalkleyK Dobrenis
Oct 7, 2004·Biochimica Et Biophysica Acta·Steven U Walkley, Kinuko Suzuki
Mar 21, 2008·Molecular Genetics and Metabolism·Douglas R MartinHenry J Baker
Feb 24, 2009·Brain Research·Jill M WeimerDavid A Pearce
Feb 8, 2003·Acta Paediatrica. Supplement·M HaskinsU Giger
Feb 11, 1998·Brain Pathology·S U Walkley
Apr 24, 1995·Biochimica Et Biophysica Acta·V Gieselmann

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