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Volume 8 Supplement 1

Ubiquitin-Proteasome System in Disease Part 1

Reviews

Edited by Edited by John Mayer and Rob Layfield

  1. The human genome encodes several ubiquitin-like (UBL) domain proteins (UDPs). Members of this protein family are involved in a variety of cellular functions and many are connected to the ubiquitin proteasome s...

    Authors: Louise Madsen, Andrea Schulze, Michael Seeger and Rasmus Hartmann-Petersen
    Citation: BMC Biochemistry 2007 8(Suppl 1):S1
  2. A functional ubiquitin proteasome system is essential for all eukaryotic cells and therefore any alteration to its components has potential pathological consequences. Though the exact underlying mechanism is u...

    Authors: Burkhardt Dahlmann
    Citation: BMC Biochemistry 2007 8(Suppl 1):S3
  3. Multiple steps in the RANK-NF-κB signalling pathway are regulated by ubiquitylation. Mutations affecting different components of this pathway, including the ubiquitin binding p62 signalling adapter protein, ar...

    Authors: Robert Layfield and Barry Shaw
    Citation: BMC Biochemistry 2007 8(Suppl 1):S5
  4. In a simplified view, members of the HECT E3 family have a modular structure consisting of the C-terminal HECT domain, which is catalytically involved in the attachment of ubiquitin to substrate proteins, and ...

    Authors: Martin Scheffner and Olivier Staub
    Citation: BMC Biochemistry 2007 8(Suppl 1):S6
  5. Fanconi anemia (FA) is a rare genetic disorder characterized by aplastic anemia, cancer/leukemia susceptibility and cellular hypersensitivity to DNA crosslinking agents, such as cisplatin. To date, 12 FA gene ...

    Authors: Céline Jacquemont and Toshiyasu Taniguchi
    Citation: BMC Biochemistry 2007 8(Suppl 1):S10
  6. CF is an inherited autosomal recessive disease whose lethality arises from malfunction of CFTR, a single chloride (Cl-) ion channel protein. CF patients harbor mutations in the CFTR gene that lead to misfolding o...

    Authors: Emma L Turnbull, Meredith FN Rosser and Douglas M Cyr
    Citation: BMC Biochemistry 2007 8(Suppl 1):S11
  7. Though Alzheimer's disease (AD) is a syndrome with well-defined clinical and neuropathological manifestations, an array of molecular defects underlies its pathology. A role for the ubiquitin proteasome system ...

    Authors: Sudarshan C Upadhya and Ashok N Hegde
    Citation: BMC Biochemistry 2007 8(Suppl 1):S12
  8. Parkinson's disease (PD) is the most common neurodegenerative movement disorder. Although a subject of intense research, the etiology of PD remains poorly understood. Recently, several lines of evidence have i...

    Authors: Kah-Leong Lim and Jeanne MM Tan
    Citation: BMC Biochemistry 2007 8(Suppl 1):S13