Protein farnesylation and disease

Giuseppe Novelli, Maria Rosaria D'Apice

Research output: Contribution to journalArticle

27 Citations (Scopus)

Abstract

Prenylation consists of the addition of an isoprenoid group to a cysteine residue located near the carboxyl terminal of a protein. This enzymatic posttranslational modification is important for the maturation and processing of proteins. Both processes are necessary to mediate protein-protein and membrane-protein associations, in addition to regulating the localisation and function of proteins. The severe phenotype of animals deficient in enzymes involved in both prenylation and maturation highlights the significance of these processes. Moreover, alterations in the genes coding for isoprenylated proteins or enzymes that are involved in both prenylation and maturation processes have been found to be the basis of severe human diseases, such as cancer, neurode-generative disorders, retinitis pigmentosa, and premature ageing syndromes. Recent studies on isoprenylation and postprenylation processing in pathological conditions have unveiled surprising aspects of these modifications and their roles in different cellular pathways. The identification of these enzymes as therapeutic targets has led researchers to validate their effects in vitro and in vivo as antitumour or antiageing agents. This review attempts to summarise the basic aspects of protein isoprenylation and postprenylation, integrating our data with that observed in other studies to provide a comprehensive scenario of progeroid syndromes and the therapeutic avenues.

Original languageEnglish
Pages (from-to)917-926
Number of pages10
JournalJournal of Inherited Metabolic Disease
Volume35
Issue number5
DOIs
Publication statusPublished - Sep 2012

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Protein Prenylation
Prenylation
Proteins
Enzymes
Premature Aging
Retinitis Pigmentosa
Terpenes
Post Translational Protein Processing
Cysteine
Membrane Proteins
Research Personnel
Phenotype
Therapeutics
Genes

ASJC Scopus subject areas

  • Genetics(clinical)
  • Genetics

Cite this

Protein farnesylation and disease. / Novelli, Giuseppe; D'Apice, Maria Rosaria.

In: Journal of Inherited Metabolic Disease, Vol. 35, No. 5, 09.2012, p. 917-926.

Research output: Contribution to journalArticle

Novelli, Giuseppe ; D'Apice, Maria Rosaria. / Protein farnesylation and disease. In: Journal of Inherited Metabolic Disease. 2012 ; Vol. 35, No. 5. pp. 917-926.
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