Disorders of protein biogenesis and stability

John F. Anderson, Efraín Siller, José M. Barral

    Research output: Contribution to journalArticle

    5 Citations (Scopus)

    Abstract

    The great diversity of structural conformations available to proteins allows this class of molecules to carry out the vast majority of biochemical functions in the cell. In order to function adequately, proteins must be synthesized, folded/assembled and degraded with great temporal and spatial accuracy. Precise coordination of multiple processes, including ribosome assembly and movement along mRNA, charging and recycling of tRNAs, recruitment and action of molecular chaperones, and tight control of the degradation machinery is essential to create and maintain a stable proteome. It has become recently evident that even slight errors in any of these processes may lead to disease states. Accordingly, increasing numbers of human diseases have been identified that are due to mutations in genes encoding proteins involved in this so-called "protein quality control". Since these processes are essential for the production and maintenance of the entire proteome of the cell, the deleterious effects of these mutations often extend far beyond the faulty gene. This review provides an overview of human disorders caused by defects in mechanisms underlying protein biogenesis and stability.

    Original languageEnglish (US)
    Pages (from-to)110-121
    Number of pages12
    JournalProtein and Peptide Letters
    Volume18
    Issue number2
    DOIs
    StatePublished - Feb 2011

    Fingerprint

    Protein Stability
    Proteome
    Proteins
    Transfer RNA Aminoacylation
    Mutation
    Molecular Chaperones
    Recycling
    Ribosomes
    Gene encoding
    Quality Control
    Transfer RNA
    Maintenance
    Machinery
    Quality control
    Conformations
    Messenger RNA
    Genes
    Degradation
    Defects
    Molecules

    Keywords

    • Chaperone
    • Degradation
    • Mutation
    • Protein folding
    • Protein synthesis
    • Translation

    ASJC Scopus subject areas

    • Biochemistry
    • Structural Biology

    Cite this

    Disorders of protein biogenesis and stability. / Anderson, John F.; Siller, Efraín; Barral, José M.

    In: Protein and Peptide Letters, Vol. 18, No. 2, 02.2011, p. 110-121.

    Research output: Contribution to journalArticle

    Anderson, JF, Siller, E & Barral, JM 2011, 'Disorders of protein biogenesis and stability', Protein and Peptide Letters, vol. 18, no. 2, pp. 110-121. https://doi.org/10.2174/092986611794475075
    Anderson, John F. ; Siller, Efraín ; Barral, José M. / Disorders of protein biogenesis and stability. In: Protein and Peptide Letters. 2011 ; Vol. 18, No. 2. pp. 110-121.
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