Volume 8 Supplement 1

Ubiquitin-Proteasome System in Disease Part 1


Edited by Edited by John Mayer and Rob Layfield

  1. Review

    Ubiquitin domain proteins in disease

    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...

    Louise Madsen, Andrea Schulze, Michael Seeger and Rasmus Hartmann-Petersen

    BMC Biochemistry 2007 8(Suppl 1):S1

    Published on: 22 November 2007

  2. Review

    Role of proteasomes in disease

    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...

    Burkhardt Dahlmann

    BMC Biochemistry 2007 8(Suppl 1):S3

    Published on: 22 November 2007

  3. Review

    Role of the ubiquitin proteasome system in renal cell carcinoma

    Renal cell carcinoma (RCC) accounts for approximately 2.6% of all cancers in the United States. While early stage disease is curable by surgery, the median survival of metastatic disease is only 13 months. In ...

    Paul G Corn

    BMC Biochemistry 2007 8(Suppl 1):S4

    Published on: 22 November 2007

  4. Review

    Ubiquitin-mediated signalling and Paget's disease of bone

    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...

    Robert Layfield and Barry Shaw

    BMC Biochemistry 2007 8(Suppl 1):S5

    Published on: 22 November 2007

  5. Review

    HECT E3s and human disease

    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 ...

    Martin Scheffner and Olivier Staub

    BMC Biochemistry 2007 8(Suppl 1):S6

    Published on: 22 November 2007

  6. Review

    Role of the ubiquitin system and tumor viruses in AIDS-related cancer

    Tumor viruses are linked to approximately 20% of human malignancies worldwide. This review focuses on examples of human oncogenic viruses that manipulate the ubiquitin system in a subset of viral malignancies;...

    Julia Shackelford and Joseph S Pagano

    BMC Biochemistry 2007 8(Suppl 1):S8

    Published on: 22 November 2007

  7. Review

    The Fanconi anemia pathway and ubiquitin

    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 ...

    Céline Jacquemont and Toshiyasu Taniguchi

    BMC Biochemistry 2007 8(Suppl 1):S10

    Published on: 22 November 2007

  8. Review

    The role of the UPS in cystic fibrosis

    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...

    Emma L Turnbull, Meredith FN Rosser and Douglas M Cyr

    BMC Biochemistry 2007 8(Suppl 1):S11

    Published on: 22 November 2007

  9. Review

    Role of the ubiquitin proteasome system in Alzheimer's disease

    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 ...

    Sudarshan C Upadhya and Ashok N Hegde

    BMC Biochemistry 2007 8(Suppl 1):S12

    Published on: 22 November 2007

  10. Review

    Role of the ubiquitin proteasome system in Parkinson's disease

    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...

    Kah-Leong Lim and Jeanne MM Tan

    BMC Biochemistry 2007 8(Suppl 1):S13

    Published on: 22 November 2007

  11. Review

    Patented small molecule inhibitors in the ubiquitin proteasome system

    Deregulation of the ubiquitin proteasome system (UPS) has been implicated in the pathogenesis of many human diseases, including cancer and neurodegenerative disorders. The recent approval of the proteasome inh...

    Philippe Guédat and Frédéric Colland

    BMC Biochemistry 2007 8(Suppl 1):S14

    Published on: 22 November 2007