Mutations that influence the secretory path in animal cells

Glucocorticoid-regulated glycoprotein maturation in wild-type and mutant rat cell lines.

“…For example, in active or carrier-mediated pathways, vesicle or organelle-associated "sorting receptors" have been postulated to bind and segre- (2,25), presumably by recognizing features of the polypeptide backbone or of the oligosaccharide side chains, as with the mannose-6-phosphate receptors involved in targeting lysosomal hydrolases (10,56). Previous work of others suggests that some of these processes may be amenable to genetic dissection in cultured cells (57,59). For example, Kabat and coworkers have isolated mutants defective in processing Friend murine leukemia virus or Friend spleen focus-forming virus envelope glycoproteins (16,49), and Zilberstein et al (65) have recovered variants that fail to produce cell surfaceassociated and lipid-modified vesicular stomatitis virus gene products.…”

“…p OLYPEPTIDES destined to be membrane associated or secreted undergo a series of specific processing and compartmentalization reactions during and after translation (21,44,50,52,57,62,63). Cell biological and genetic data (1,11,17,48,51,52,59) suggest that shuttle vesicles selectively transport polypeptides from the rough endoplasmic reticulum into the Golgi stacks, through the Golgi cisternae and to their final intra-or extracellular locations, apparently subjecting the polypeptides to an ordered array of modifications. For example, different Golgi cisternae appear to contain specific glycosyltransferases that modify glycoprotein substrates during passage through individual compartments (7,8,10,21,44).…”

Inhibitors of protein glycosylation and glycoprotein processing in viral systems

“…(For a review see Kornfeld and Kornfeld, 1985). secretory pathway of cells can be used to equip glycoproteins with altered oligosaccharide structures (Tartakoff, 1983a).…”

Transport to cell surface of intestinal sucrase-isomaltase is blocked in the Golgi apparatus in a patient with congenital sucrase-isomaltase deficiency.

“…Current thinking assumes that this transport is mediated by receptor-like proteins recognizing signals (2,6) in the proteins to be sorted out and that membrane vesicles are involved in the intercompartmental traffic (7). Among the approaches used for dissecting the sequence of events and gene products that act at discrete steps, the analysis of naturally occurring and induced mutant phenotypes is particularly promising (8).…”