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  1. Article ; Online: Spectral properties and mechanisms that underlie autofluorescent accumulations in Batten disease.

    Seehafer, Sabrina S / Pearce, David A

    Biochemical and biophysical research communications

    2009  Volume 382, Issue 2, Page(s) 247–251

    Abstract: 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 ... ...

    Abstract 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 in normal control lymphoblasts we have determined that disruptions in microtubule assembly and non-muscle myosin II function results in accumulation of lysosomal AFSM. Interestingly, inhibition of autophagy did not result in AFSM. We conclude that cellular disturbances outside the lysosome in addition to compromised function of this organelle can result in accumulation of lysosomal AFSM in NCLs and possibly as a result of cellular aging.
    MeSH term(s) Adolescent ; Adult ; Cellular Senescence ; Child ; Child, Preschool ; Female ; Fluorescence ; Humans ; Lysosomes/metabolism ; Male ; Microtubules/metabolism ; Myosin Type II/metabolism ; Neuronal Ceroid-Lipofuscinoses/metabolism ; Neuronal Ceroid-Lipofuscinoses/pathology ; Spectrometry, Fluorescence
    Chemical Substances Myosin Type II (EC 3.6.1.-)
    Language English
    Publishing date 2009-02-25
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural
    ZDB-ID 205723-2
    ISSN 1090-2104 ; 0006-291X ; 0006-291X
    ISSN (online) 1090-2104 ; 0006-291X
    ISSN 0006-291X
    DOI 10.1016/j.bbrc.2009.02.099
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 116: Show issues Location:
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  2. Article: You say lipofuscin, we say ceroid: defining autofluorescent storage material.

    Seehafer, Sabrina S / Pearce, David A

    Neurobiology of aging

    2006  Volume 27, Issue 4, Page(s) 576–588

    Abstract: Accumulation of intracellular autofluorescent material or "aging pigment" has been characterized as a normal aging event. Certain diseases also exhibit a similar accumulation of intracellular autofluorescent material. However, autofluorescent storage ... ...

    Abstract Accumulation of intracellular autofluorescent material or "aging pigment" has been characterized as a normal aging event. Certain diseases also exhibit a similar accumulation of intracellular autofluorescent material. However, autofluorescent storage material associated with aging and disease has distinct characteristics. Lipofuscin is a common term for aging pigments, whereas ceroid is used to describe pathologically derived storage material, for example, in the neuronal ceroid lipofuscinoses (NCLs). NCLs are a family of neurodegenerative diseases that are characterized by an accumulation of autofluorescent storage material (ceroid) in the lysosome, which has been termed "lipofuscin-like". There have been many studies that describe this autofluorescent storage material, but what is it? Is this accumulation lipofuscin or ceroid? In this review we will try to answer the following questions: (1) What is lipofuscin and ceroid? (2) What contributes to the accumulation of this storage material in one or the other? (3) Does this material have an effect on cellular function? Studying parallels between the accumulation of lipofuscin and ceroid may provide insight into the biological relevance of these phenomena.
    MeSH term(s) Aging/physiology ; Animals ; Autophagy/physiology ; Ceroid/analysis ; Humans ; Lipofuscin/analysis ; Lysosomes/metabolism ; Models, Biological ; Nervous System Diseases/metabolism ; Neuronal Ceroid-Lipofuscinoses/genetics ; Neuronal Ceroid-Lipofuscinoses/metabolism ; Oxidation-Reduction
    Chemical Substances Ceroid ; Lipofuscin
    Language English
    Publishing date 2006-04
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Review
    ZDB-ID 604505-4
    ISSN 1558-1497 ; 0197-4580
    ISSN (online) 1558-1497
    ISSN 0197-4580
    DOI 10.1016/j.neurobiolaging.2005.12.006
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 1685: Show issues Location:
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    Zs.MG 10: Show issues

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  3. Article ; Online: Immunosuppression alters disease severity in juvenile Batten disease mice.

    Seehafer, Sabrina S / Ramirez-Montealegre, Denia / Wong, Andrew Ms / Chan, Chun-Hung / Castaneda, Julian / Horak, Michael / Ahmadi, Sarah M / Lim, Ming J / Cooper, Jonathan D / Pearce, David A

    Journal of neuroimmunology

    2010  Volume 230, Issue 1-2, Page(s) 169–172

    Abstract: Autoantibodies to brain proteins are present in Juvenile Neuronal Ceroid Lipofuscinosis (Batten disease) patients and in the Cln3-/- mouse model of this disease, suggesting an autoimmune component to pathogenesis. Using genetic or pharmaceutical ... ...

    Abstract Autoantibodies to brain proteins are present in Juvenile Neuronal Ceroid Lipofuscinosis (Batten disease) patients and in the Cln3-/- mouse model of this disease, suggesting an autoimmune component to pathogenesis. Using genetic or pharmaceutical approaches to attenuate this immune response in Cln3-/- mice, we demonstrate decreased neuroinflammation, decreased deposition of immunoglobulin G in the brain and protection of vulnerable neuron populations. Moreover, immune suppression results in a significant improvement in motor performance providing for the first plausible therapeutic approach for juvenile Batten disease.
    MeSH term(s) Animals ; Autoantibodies/drug effects ; Autoantibodies/immunology ; Blotting, Western ; Brain/drug effects ; Brain/pathology ; Cell Count ; Disease Models, Animal ; Immunohistochemistry ; Immunosuppression ; Immunosuppressive Agents/pharmacology ; Male ; Membrane Glycoproteins/genetics ; Mice ; Mice, Inbred C57BL ; Mice, Knockout ; Molecular Chaperones/genetics ; Motor Skills/drug effects ; Mycophenolic Acid/analogs & derivatives ; Mycophenolic Acid/pharmacology ; Neuronal Ceroid-Lipofuscinoses/drug therapy ; Neuronal Ceroid-Lipofuscinoses/immunology ; Neuronal Ceroid-Lipofuscinoses/pathology ; Neurons/drug effects ; Neurons/pathology
    Chemical Substances Autoantibodies ; CLN3 protein, mouse ; Immunosuppressive Agents ; Membrane Glycoproteins ; Molecular Chaperones ; Mycophenolic Acid (HU9DX48N0T)
    Language English
    Publishing date 2010-10-09
    Publishing country Netherlands
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 8335-5
    ISSN 1872-8421 ; 0165-5728
    ISSN (online) 1872-8421
    ISSN 0165-5728
    DOI 10.1016/j.jneuroim.2010.08.024
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 1646: Show issues Location:
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