Ribitol-phosphate—a newly identified posttranslational glycosylation unit in mammals: structure, modification enzymes and relationship to human diseases

High-throughput profiling of posttranslational modification enzymes by phage display.

Phage display has been used as a high-throughput platform for identifying proteins or peptides with desired binding or catalytic activities from a complex proteome. Recently, phage display has been applied to profile the catalytic activities of posttranslational modification (PTM) enzymes. Here, we highlight recent work elucidating the downstream targets of PTM enzymes by phage display, includi...

Genetic Diseases Associated with Protein Glycosylation Disorders in Mammals

Human diseases with underlying defects in chromatin structure and modification.

Chromatin structure is important for regulating gene expression and for the proper condensation and segregation of chromosomes during cell division. Several human genetic diseases have been found to be due to mutations in genes producing proteins known or suspected to be involved in maintaining or modifying chromatin structure. Here we describe these 'chromatin diseases' and review what is know...

Relationship between the newly identified bile acid binder and bile acid oxidoreductases in human liver.

Gel filtration fractions of human hepatic cytosol obtained from an autopsy liver were examined for elution of bile acid oxidoreductases. Several enzymes including 3 alpha-hydroxysteroid dehydrogenase (3 alpha-HSD), 3 beta-hydroxysteroid dehydrogenase (3 beta-HSD), 3-ketosteroid reductase, and dihydrodiol dehydrogenase eluted mainly in the 30,000-40,000 Mr fractions known to contain the newly id...

Posttranslational modification and quality control.

Protein quality control functions to minimize the level and toxicity of misfolded proteins in the cell. Protein quality control is performed by intricate collaboration among chaperones and target protein degradation. The latter is performed primarily by the ubiquitin-proteasome system and perhaps autophagy. Terminally misfolded proteins that are not timely removed tend to form aggregates. Their...