2.8.2.5 additional information CS-deficient sog9 cells, derived from gro2C cells partially resistant to HSV-1 infection and defective in the expression of heparan sulfate because of a splice site mutation in the EXT1 gene encoding the HS-synthesizing enzyme, have a defect in the expression of C4ST-1 in addition to EXT1, decrease in the amount of chondroitin sulfate in sog9 cells is the result of a reduction in the length of chondroitin sulfate chains, glycosyltransferases participating in the biosynthesis of chondroitin sulfate are not affected in sog9 cells, rescue of sog9 cells only by introducing C4ST-1 674724 2.8.2.5 additional information deletion of N-glycosylation sites, loss of the N-glycosylation site at the C-terminus reduces activity by 80% 660998 2.8.2.5 additional information enzyme knockout by siRNA 761061 2.8.2.5 additional information functional knockdown of C4ST-1 with specific antisense morpholino-oligonucleotides leads to a marked decrease in the 4-O-sulfation and amount of chondroitin sulfate in the embryos. Consistent with the preferential expression in the rostrocaudal axis, C4ST-1 morphants display morphological defects exemplified by a ventrally bent trunk and a curled and/or kinky tail, largely due to misregulated myotomal myod expression, implying perturbation of axial muscle differentiation in somites. Rescue of morphological phenotypes of the MO1-injected embryos by injection with zebrafish C4ST-1 RNA, phenotypes, overview 702127 2.8.2.5 additional information gene-trap mutation in bone morphogenic protein-induced enzyme causes severe chondrodysplasia characterised by disorganised cartilage growth plate as well as specific alterations in the orientation of chondrocyte columns. Phenotype is associated with chondroitin sulfation imbalance, mislocalization of chondroitin sulfate and imbalance of apoptotic signals 661565 2.8.2.5 additional information reconstitution of the chondroitin biosynthesis pathway in a recombinant Bacillus subtilis strain using sucrose as carbon source, method optimization, overview. Specific sulfation transformation systems are successfully constructed and optimized by combining the purified aryl sulfotransferase IV (ASST IV, EC 2.8.2.1), chondroitin 4-sulfotransferase (C4ST) and chondroitin 6-sulfotransferase (C6ST, EC 2.8.2.17). Chondroitin sulfate A and C are enzymatically transformed from chondroitin at conversion rates of 98% and 96%, respectively. The ASST IV enzyme is used for PAPS regeneration. Purification of CS disaccharides by anion exchange chrmatography and NMR analysis of the composition of CS disaccharides 760752 2.8.2.5 additional information translocations t(12,14)(q23,q32) in a patient with B-cell chronic lymphocytic leukemia result in expression of two truncated forms of enzyme protein, resulting in disturbance of the cellular distribution of enzyme, and further in deregulation of a chondroitin-sulfate dependent pathway specific to the hematopoietic lineage 662997 2.8.2.5 R186A the mutant fails to bind 3'-phosphoadenylyl sulfate 721529