Cystic fibrosis as a disease of misprocessing of the cystic fibrosis transmembrane conductance regulator glycoprotein

Article Abstract:

Cystic fibrosis involves mutations in the gene for CFTR (Cystic Fibrosis Transmembrane Conductance Regulator). Usually there is at least one CFTR allele that causes synthesis of a protein improperly folded or assembled. Biosynthetic quality control mechanisms prevent the product from maturing or moving beyond the endoplasmic reticulum. The proteins could be functional if they were able to reach the cell surface. A method for promoting maturation of misfolded proteins could be helpful in treating cystic fibrosis and other diseases.

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Multiple proteolytic systems, including the proteasome, contribute to CFTR processing

Article Abstract:

Degradation of cystic fibrosis transmembrane conductance regulator (CFTR) is affected by inhibitors of cytosolic proteasome, such as lactacystin and some peptide aldehydes. Degradation is stopped by MG-132, a peptide aldehyde, by blocking ATP-dependent change of the wild-type precursor to to the native folded form. Proteasomal degradation of integral membrane proteins, such as CFTR, occurs during their maturation within endoplasmic reticulum.

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Foxo transcription factors induce the atrophy-related ubiquitin ligase Atrogin-1 and cause skeletal muscle atrophy

Article Abstract:

A study to show that in cultured myotubes undergoing atrophy, the activity of the PI3K/AKT pathway decreases, leading to activation of Foxo transcription factors and the ubiquitin ligase, atrogin-1 induction is presented. The findings show that fork-head factors play a critical role in the development of muscle atrophy, and inhibition of Foxo factors is an attractive approach to combat muscle wasting.

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