Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.1.6.4 (chondroitinase)
 2,039 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Synovial fluid was collected from the superior articular cavity of the temporomandibular joint in patients with unilateral internal derangement and joint pain whose contralateral joint was healthy. Glycosaminoglycans were liberated by digestion with pronase E, and precipitated with cetylpyridinium chloride and ethanol. Unsaturated disaccharide isomers of chondroitin sulfate, obtained following chondroitinase ACII digestion, were analyzed by high-performance liquid chromatography. Analytic data indicated that deltaDi-0S and deltaDi-6S were often found in chondroitin sulfate from the fluid of the diseased joints. The amounts of deltaDi-0S and deltaDi-6S differed significantly between synovial fluid samples from the diseased and healthy joints. Comparison of the relative proportions of the unsaturated disaccharides in the synovial fluid with previously reported values for several tissues, indicated that the chondroitin sulfate originated from articular cartilage, with possibly some contributions from soft connective tissues and serum present in the synovial fluid. These results suggest that chondroitin sulfate in the synovial fluid provides a useful indicator of the degree of internal derangement of the temporomandibular joint.
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PMID:Chondroitin sulfate isomers in synovial fluid of healthy and diseased human temporomandibular joints. 939 5

Mucopolysaccharidosis IVA (MPS IVA) is one of the lysosomal storage diseases. It is caused by the deficiency of N-acetylgalactosamine-6-sulfate sulfatase. Deficiency of this enzyme leads to accumulation of the specific glycosaminoglycans keratan sulfate and chondroitin-6-sulfate. This accumulation has a direct impact on cartilage and bone development, resulting in systemic skeletal dysplasia. There is no curative therapy for this skeletal dysplasia. This report describes long-term therapeutic efficacy in a 15-year-old boy with a severe form of MPS IVA who received successful allogeneic bone marrow transplantation (BMT) from his HLA-identical carrier sister. The level of the GALNS enzyme in the recipient's lymphocytes reached almost half of normal level within two years after BMT. For the successive 9+ years post-BMT, GALNS activity in his lymphocytes maintained the same level as the donor's, and the level of urinary uronic acid was reduced. Lumbar bone mineral density increased around 50% one year later post-BMT and was kept consistent. Radiographs showed that the figures of trochanter major and minor appeared, while the epiphyseal dysplasia in the femoral cap was almost unchanged. Loud snoring and apnea disappeared. Vital capacity increased to around 20% for the first two years and was maintained. Activity of daily life (ADL) was improved in work/study efficacy, respiratory status, sleep, joint pain, and frequency of infection. In conclusion, the long-term study of hematopoetic stem cell transplantation has shown clinical improvements in respiratory function, radiograph findings, ADL, and biochemical findings, suggesting that it is a potential therapeutic option for patients with MPS IVA.
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PMID:Long-term therapeutic efficacy of allogenic bone marrow transplantation in a patient with mucopolysaccharidosis IVA. 2559 92