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The genetics of frontotemporal lobar degeneration.

The genetics of frontotemporal lobar degeneration. Research Abstract Details 

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  • The genetics of frontotemporal lobar degeneration. Abstract Text:

    rosa rademakersRosa Rademakers,mike huttonMike Hutton,

    The clinical disorders associated with frontotemporal lobar degeneration (FTLD) are increasingly recognized as an important cause of early-onset dementia. Patients usually present with progressive changes in personality, behavior, or language, progressing to general cognitive impairment and ultimately death. In the past decade, improved clinical and histopathologic characterization uncovered extensive heterogeneity, and multiple clinical and pathologic FTLD subtypes were defined. Simultaneously, the discovery of four causal FTLD genes emphasized the genetic complexity associated with FTLD. More recently, the field of FTLD has gained increased attention as a result of two major findings. First, mutations in the progranulin gene (PGRN) were recognized as a major cause of FTLD with ubiquitin-positive and tau-negative inclusions (FTLD-U), and subsequently the TAR DNA-binding protein-43 (TDP-43) was identified as a key protein within the ubiquitinated inclusions in FTLD-U and amyotrophic lateral sclerosis (ALS). In this report, we outline the progress made in the study of the genetic etiologies and neuropathologic substrates in FTLD.

    The genetics of frontotemporal lobar degeneration. Publishing Authors By Initials

    r rademakersR Rademakers,m huttonM Hutton,

    For similar proteins: nerve tissue proteins research abstracts see: proteins: nerve tissue proteins research

    PUBMED ID PMID:

    MEDLINE DATE:

    The genetics of frontotemporal lobar degeneration. Journal Published:

    PUBLICATION TYPE: Review

    Journal: Current neurology and neuroscience reports

    VOLUME: 7

    Page Numbers: 434-42

    Journal Abbreviation:

    ISSN: 1528-4042

    DAY: 26

    MONTH: Sep

    YEAR: 2007

    The genetics of frontotemporal lobar degeneration. Information

    Number of References: 73

    LANGUAGE: eng

    NlmUniqueID: 100931790

    The genetics of frontotemporal lobar degeneration. Keywords Mesh Terms:

    KEYWORDS: Nerve Tissue Proteins

    MESH TERMS: genetics

    Chemical & Substance for Abstract: The genetics of frontotemporal lobar degeneration. Information

    Substance Name: Adenosine Triphosphatases

    Registry Number: EC 3.6.1.-

    Grant and Affiliation Information for The genetics of frontotemporal lobar degeneration.

    AFFILIATION: Department of Neuroscience, Mayo Clinic College of Medicine, 4500 San Pablo Road, Jacksonville, FL 32224, USA. rademakers.rosa@mayo.edu

    Country: United States

    United States Research PublicationUnited States Research Publication

    AGENCY: United States NIA

    GRANT: R01 AG026251

    ACRONYM: AG

    MEDLINETA: Curr Neurol Neurosci Rep

    REFSOURCE:

    DATABASENAME:

    ACCESSION NUMBER:

    Number Hits: 0

    The genetics of frontotemporal lobar degeneration Related Publications

     

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