Protein aggregation is a hallmark of a large and diverse number of conformational diseases. Molecular chaperones of the Hsp40 family (Escherichia coli DnaJ homologs) recognize misfolded disease proteins and suppress the accumulation of toxic protein species. Type I Hsp40s are very potent at suppressing protein aggregation and facilitating the refolding of damaged proteins. Yet, the molecular me...

congenital recessive myotonia is a rare genetic disorder caused by mutations in clcn1, which codes for the main skeletal muscle chloride channel clc-1. more than 120 mutations have been found in this gene. the main feature of this disorder is muscle membrane hyperexcitability. here, we report a 59-year male patient suffering from congenital myotonia. he had transient generalized myotonia, which...

The carcinoembryonic antigen (CEA) gene family belongs to the immunoglobulin supergene family and can be divided into two main subgroups based on sequence comparisons. In humans it is clustered on the long arm of chromosome 19 and consists of approximately 20 genes. The CEA subgroup genes code for CEA and its classical crossreacting antigens, which are mainly membrane-bound, whereas the other s...

Hsp90 is an abundant and ubiquitous protein involved in a diverse array of cellular processes. Mechanistically we understand little of the apparently complex interactions of this molecular chaperone. Recently, progress has been made in assigning some of the known functions of hsp90, such as nucleotide binding and peptide binding, to particular domains within the protein. We used fragments of hs...

DnaJ proteins are essential co-chaperones involved in abiotic and biotic stress responses. Arabidopsis AtDjA3 gene encodes a molecular co-chaperone of 420 amino acids, which belongs to the J-protein family. In this study, we report the functional characterization of the AtDjA3 gene using the Arabidopsis knockout line designated j3 and the 35S::AtDjA3 overexpression lines. Loss of AtDjA3 functio...

member of the ras homolog gene family.

Neuroblastoma is one of the most intensely studied solid malignancies that affect children (Maris & Matthay, 1999). These tumours are heterogeneous biologically and clinically. One subset of neuroblastoma is susceptible to spontaneous apoptosis with little or no therapy and another subset differentiates over time, but most of these tumours are difficult to cure with current treatments. A relati...