PMID: 8971792Nov 22, 1996Paper

Evidence that lysosomal storage of proteolipids is a cell autonomous process in the motor neuron degeneration (mnd) mouse, a model of neuronal ceroid lipofuscinosis

Neuroscience Letters
R D LipmanR T Bronson

Abstract

The motor neuron degeneration (mnd) mouse has been documented to accumulate proteolipid and thus is a model of neuronal ceroid lipofuscinosis [Dunn, W.A., Raizada, M.K., Vogt, E.S. and Brown, E.A., Int. J. Dev. Neurosci., 12 (1994) 185-196; Faust, J.R., Rodman, J.S., Daniel, P.F., Dice, J.F. and Bronson, R.T., J. Biol. Chem., 269 (1994) 10150-10155]. While accumulation of proteolipid in the hippocampus of chimeric mice composed of mnd and +/+ cells was found to be proportional to the contribution of mnd in the brain, accumulation within individual cells was the same for cells from chimeric and age-matched mnd mice. Bone marrow transplantation was used to altering the milieu of circulating factors to determine whether this might modify the disease phenotype in mnd mice. Transplantation of bone marrow in neonatal or young mice did not reduce the age-associated accumulation of proteolipid within hippocampal neurons. The results of these experiments indicate that mnd results in a cell autonomous defect.

References

Nov 1, 1982·Somatic Cell Genetics·R D Lipman, A L Muggleton-Harris
Jun 5, 1995·American Journal of Medical Genetics·V J WestlakeW Krivit
Jun 1, 1994·International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience·W A DunnE A Brown
Apr 13, 1996·Lancet·P B MunroeI Jarvela

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Citations

Jul 1, 1997·Brain & Development·B C KieseierH H Goebel
Feb 4, 2012·Journal of Neuroscience Research·Paolo BiginiGiambattista Bonanno
Mar 9, 1999·Neurobiology of Disease·S E Mole
May 26, 1998·American Journal of Medical Genetics·R T BronsonJ R Faust
Mar 7, 2003·Bone Marrow Transplantation·C PetersUNKNOWN Working Party on Inborn Errors, European Bone Marrow Transplant Group
Feb 11, 1998·Brain Pathology·H H Goebel, J D Sharp
May 31, 2021·Journal of Neuroscience Methods·Megan Lynn MorganPeter K Stys

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Batten Disease is a group of nervous system disorders known as neuronal ceroid lipofuscinosis. This feed focuses on neurobiological and neuropathological aspects of this disease.