Aberrant protein

Anomalous ubiquitin-B molecules are also present in the brains of control subjects, probably indicating a preneurodegenerative stage. The aberrant proteins show a cellular colocalization compatible with alterations at the transcriptional level or dysfunctional posttranscriptional RNA editing (138). 

Knowles, S.E., and Ballard, F.J. (1976). Selective control of the degradation of normal and aberrant proteins in Reuber H35 hepatoma cells. Biochem J. 156, 609-617. 

It has long been suggested that ROS may play causal roles in these diseases and in the production of the aberrant protein molecules (de Arriba et al., 2006). In addition, protein damage inflicted by RNS has also been suggested. As discussed above, carnosine has been shown to possess antioxidant activity and also to react with RNS as well, hence the molecule has the potential to be considered as a theraupeutic agent (Calabrese et al., 2008). 

Hipkiss, A. R. (2003). Do developmental-related changes in constitutive proteolysis affect aberrant protein accumulation and generation of the aged phenotype Mech. Ageing Dev. 124, 575-579. 

The RS04 cells can react with anti-Eas mAb, but are not killed, suggesting incomplete trimerization between aberrant mEas and intact mFas. Recently, three-dimensional analysis using computer modeling has provided new insights into the structure and function of aberrant proteins (H3). 

L-Canavanine and L-canaline are non-protein amino acids of certain leguminous plants, that function as protective allelochemicals. L-Canavanine is incorporated into de novo synthesized proteins in place of arginTne there is suggestive evidence that formation of such anomalous proteins figures significantly in canavanine s adverse biological effects. Canavanine, however, does not appear to inhibit overall protein synthesis. Thus, an important basis for canavanine s antimetabolic properties resides in the sustained production of biologically aberrant proteins. 

Endotoxins are found in some bacterial sources, such as E. coli. For other products they are considered a contaminant that should not be present and can be controlled by adherence to good manufacturing practices (GMPs). Nucleic acids, once considered a significant risk, are now thought of as cellular impurities, and their removal should be validated [2,3]. Proteins that pose a potential risk (e.g., immunogenicity) include host cell proteins, aberrant protein product, proteins used in cell culture, and those associated with the process (e.g., protein A affinity ligands or nucleases employed to reduce viscosity). 

Ovadi,)., Orosz, F., Hollan, S. Functional aspects of cellular microcompartmenta-tion in the development of neurodegeneration mutation induced aberrant protein-protein associations Mol. Cell. Biochem. 2004, 256/257 83-93. 

The Importance of Normal and Aberrant Protein Folding in Biology... 

Cockerill (4) prepared protein tyrosine kinase inhibitors consisting of anilinoquinazoline derivatives, (IV), to regulate aberrant protein tyrosine kinase activity characterized by overexpression or mutation resulting in uncontrolled cell growth. 

Several authors have provided evidence that pathological-length Qn repeats promote aberrant protein interactions that cause defects in axonal transport (Gunawardena and Goldstein, 2005 Smith et al 2009 Schweitzer et al., 2009 Wu et al.,... 

The Qn expansion causes aberrant protein conformation, which in turn leads to altered Ca " " homeostasis (1), mitochondrial dysfunction (2), altered energy metabolism (3), and excitoxicity/oxidative stress (4). The protein containing the... 

Among the trinucleotide-expansion diseases, it is still not yet clear what accounts for the sensitivity of nervous tissue to the altered genotype and what accounts for the selective vulnerability of different brain regions among the various diseases. However, some progress is being made. A feature of the Qn-expansion diseases and An-expansion diseases, is the presence of (or propensity to form) aberrant protein deposits in affected brain regions. In this respect, these diseases are similar to the... 

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