Spectral properties and mechanisms that underlie autofluorescent accumulations in Batten disease.
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ABSTRACT:
SUBMITTER: Seehafer SS
PROVIDER: S-EPMC4123116 | biostudies-literature | 2009 May
REPOSITORIES: biostudies-literature
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Spectral properties and mechanisms that underlie autofluorescent accumulations in Batten disease.
Seehafer Sabrina S SS Pearce David A DA
Biochemical and biophysical research communications 20090225 2
Neuronal Ceroid Lipofuscinoses (NCLs) have an incidence of 1 in 12,500 live births. These devastating neurodegenerative lysosomal storage diseases are characterized by the lysosomal accumulation of autofluorescent storage material (AFSM) similar to that seen in aging cells. Using patient derived lymphoblasts from three genetically distinct NCLs we report that AFSM for each NCL has distinct spectral properties. Moreover, by using pharmacological inhibitors to disrupt various biochemical pathways ...[more]
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