Conclusion
The similar histochemical, fluorescent and ultrastructural appearance of some lipofuscins and the pigment of Batten disease imply some underlying common chemical feature. The similar chloroform—methanol solubility of the protein subunit c in Batten disease and the specific proteins accumulated in thyroid lipofuscin imply that disordered or delayed catabolism of hydrophobic protein may be that common feature.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Fearnley IM, Walker JE, Martinus RD, Jolly RD, Kirkland KB, Palmer DN (1990) The sequence of the major protein stored in ovine ceroid-lipofuscinosis is identical to that of the DCCD-reactive proteolipid mitochondrial ATP synthase.Biochem J 268: 751–758.
Goebel HH, Braak H, Seidel D, Doshi R, Marsden CD, Gullota F (1982) Morphological studies on adult neuronal ceroid-lipofuscinosis.Clin Neuropathol 1: 151–162.
Graydon RJ, Jolly RD (1984) Ceroid-lipofuscinosis (Batten disease): Sequential electrophysiologic and pathologic changes in the ovine model.Invest Ophthalmol Vis Sci 25: 294–301.
Jolly RD, Dalefield RR (1990) Lipopigments in veterinary pathology. In Porta EA, ed.Lipofuscin and Ceroid Pigments. New York: Plenum Press, 157–167.
Jolly RD, Shimada A, Döpfmer I, Slack PM, Birtles MJ, Palmer DN (1989) Ceroidlipofuscinosis: Pathogenesis and sequential neuropathological changes in the ovine model.Neuropathol Appl Neurobiol 15: 371–383.
Jolly RD, Martinus RD, Palmer DN (1992) Sheep and other animals with ceroid-lipofuscinosis: Their relevance to Batten's disease.Am J Med Genet 42: 85–94.
Martinus RD, Harper PAW, Jolly RD et al (1991) Bovine ceroid-lipofuscinosis (Batten's disease): the major component stored is the DCCD-reactive proteolipid, subunitc, of mitochondrial ATP synthase.Vet Res Commun 15: 85–94.
Mayhew IG, Jolly RD, Pickett BT, Slack PM (1985) Ceroid-lipofuscinosis (Batten's disease): Pathogenesis of blindness in the ovine model.Neuropathol Appl Neurobiol 11: 273–290.
Palmer DN, Martinus RD, Cooper SM, Midwinter GG, Reid JC, Jolly RD (1989) Ovine ceroid-lipofuscinosis: The major lipopigment protein and the lipid-binding subunit of mitochondrial ATP synthase have the same NH2 terminal sequence.J Biol Chem 264: 5736–5740.
Palmer DN, Fearnley IM, Medd SM et al (1990) Lysosomal storage of the DCCD reactive proteolipid subunit of mitochondrial ATP synthase in human and ovine ceroidlipofuscinoses. In Porta EA, ed.Lipofuscin and Ceroid Pigments. New York: Plenum Press, 211–223.
Palmer DN, Fearnley IM, Walker JE et al (1992) Mitochondrial ATP synthase subunit c storage in the ceroid-lipofuscinoses (Batten's disease).Am J Med Genet 42: 561–567.
Palmer DN, Bayliss SL, Clifton PA, Westlake VJ (1993) Storage bodies in the ceroidlipofuscinoses (Batten disease): low molecular-weight components, unusual amino acids and reconstruction of fluorescent bodies from non fluorescent components.J Inher Metab Dis 16: 292–295.
Zeman W (1974) Studies in the neuronal ceroid-lipofuscinoses.J Neuropathol Exp Neurol 33: 1–12.
Zeman W, Dyken P (1969) Neuronal ceroid-lipofuscinosis (Batten's disease): Relationship to amauratic family idiocy.Pediatrics 44: 570–583.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Jolly, R.D., Dalefield, R.R. & Palmer, D.N. Ceroid, lipofuscin and the ceroid-lipofuscinoses (Batten Disease). J Inherit Metab Dis 16, 280–283 (1993). https://doi.org/10.1007/BF00710265
Issue Date:
DOI: https://doi.org/10.1007/BF00710265