Curcumin in peripheral neuropathies

PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Curcumin treatment abrogates endoplasmic reticulum retention and aggregation-induced apoptosis associated with neuropathy-causing myelin protein zero-truncating mutants” (Am J Hum Genet. 2005 Nov;77(5):841-50. Epub 2005 Sep 30). Authors are Khajavi M, Inoue K, Wiszniewski W at al., from Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, USA. Mutations in the gene encoding myelin protein zero (MPZ), the major protein constituent of peripheral myelin, can cause the adult-onset, inherited neuropathy Charcot-Marie-Tooth disease, as well as the more severe, childhood-onset Dejerine-Sottas neuropathy and congenital hypomyelinating neuropathy. The authors examined the functional properties of MPZ-truncating proteins and they found that frameshift mutations associated with severe disease cause an intracellular accumulation of mutant proteins, primarily within the endoplasmic reticulum (ER), which induces apoptosis. Curcumin, a chemical compound derived from the curry spice tumeric, releases the ER-retained MPZ mutants into the cytoplasm accompanied by a lower number of apoptotic cells. The findings suggest that curcumin treatment is sufficient to relieve the toxic effect of mutant aggregation-induced apoptosis and may potentially have a therapeutic role in treating selected forms of inherited peripheral neuropathies. To access the full abstract of the article, click here.