What are lysosome with onion skins?

Discussion in 'Step I' started by UIUCstudent, Sep 22, 2012.

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  1. UIUCstudent

    UIUCstudent 2+ Year Member

    Mar 19, 2010
    Are lysosome with onion skins the same as zebra bodies inclusions?
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  3. Phloston

    Phloston Lifetime Donor 5+ Year Member

    Jan 17, 2012
    Osaka, Japan
    SDN Author
    According to the abstract below, zebra bodies were found in this girl's lysosomes along with GM2 ganglioside, but gene analysis likely demonstrated that she didn't have Taysach's (I've read somewhere that Taysach's mutations are due to a 4kb-insertion frameshift mutation). So the implication is that her histological findings are similar to Taysach's, but she doesn't have Taysach's.

    So, yeah, I would say zebra bodies inclusions are the onion skinning in lysosomes.

    Pediatr Neurol. 1997 Feb;16(2):141-4.
    Encephaloneuropathy with lysosomal zebra bodies and GM2 ganglioside storage.
    Strømme P, Månsson JE, Scott H, Skullerud K, Hovig T.
    Department of Pediatrics, Rikshospitalet, Oslo, Norway.
    An 11-year-old girl died of a neuronal storage disorder that clinically was characterized by failure to thrive and muscular hypotonia from birth, with the subsequent evolution of motor neuron disease, epilepsy, and dementia. A wide range of metabolic disorders, including all forms of GM2 gangliosidosis, could be excluded. Electron microscopy demonstrated neuronal zebra body inclusions, and immunohistochemistry demonstrated that GM2 ganglioside was a major constituent of the storage material. We suggest that the patient died of a lysosomal storage disease that is clinically and biochemically different from Tay-Sachs disease, Sandhoff disease, and other GM2 gangliosidoses described previously. This case also further demonstrates that significant accumulation of GM2 ganglioside, which is crucial for dendritic formation, may occur in neuronal storage diseases lacking known defects in ganglioside catabolism.

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