Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.2.1.36 (hyaluronidase)
 4,606 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An extracellular fluid phase (C(f1)), aspirated by micropuncture techniques from the hypertrophic cell zone of calcifying epiphyseal certilage, has been characterized in a calcifying system in vitro in respect to the behavior of sedimenting and supernatant fractions after high speed ultracentrifugation. To perform these tests on the starting samples of 20 nl of C(f1), macroscopic analytical methods were scaled down for the identification of relevant organic components, including hexuronic acid and proteinpolysaccharides (PPL). The mineral accretion system was designed to simulate physiologic conditions in the calcifying cartilage septa of normal rats, and the mineral used for seeding was an immature calcium phosphate similar to native cartilage mineral. Normal C(f1) or its dilutions in synthetic lymph up to 1:4 completely prevented mineral accretion in vitro. The inhibitory action was localized to the sedimented fractions after ultracentrifugation and could be destroyed by incubation with trypsin or hyaluronidase. The sediment of C(f1) contained 2 mg of hexuronic acid per ml of C(f1) and gave a strong reaction of identification for a light fraction of PPL by fluorescent antibodies to rat PPL. PPL fractions were tested in the same mineral accretion systems as C(f1) and exhibited responses similar to those of C(f1). Also, there was evidence of a mineral phase in C(f1) of normal rats, in C(f1) of rats with healing rickets, but not in C(f1) of untreated rachitic rats. These results are interpreted to indicate that certain PPLs function as an inhibitor of crystal growth at extracellular sites premonitory to calcification. Evidence for a low density inhibitor of mineral accretion was found in normal serum but not in C(f1).
 ...
PMID:Demonstration of macromolecular inhibitors of calcification and nucleational factors in fluid from calcifying sites in cartilage. 488 46

The localization of sudanophil material at dentine, the compact bone and epiphyseal cartilage plate of tibia of rat given beryllium carbonate was examined. Sudanophil material was seen at the boundary parts between dentine and widened predentine, and between widened preosseous matrix and calcified bone, but it was not seen at the area corresponding to the zone of provisional calcification. These facts suggest that the localization of sudanophil material in hard tissue of rat with Be rickets was similar to that in vitamin D deficient-induced rickets. This sudanophil material was not disappeared by the enzymes such as papain, pepsin and hyaluronidase as described in vitamin D deficient-induced rickets (Irving, 1960, 1963). Accordingly, it was suggested that the substance was not proteins and mucopolysaccharide.
 ...
PMID:Localization of sudanophil material at the sites of calcification in dentine, and the compact bone and epiphyseal cartilage plate of tibia in the rat given beryllium carbonate. 618 93

Hyaluronidases are endo-glycosidases that degrade both hyaluronan (hyaluronic acid) (HA) and chondroitin sulfates. Deficiency of hyaluronidase activity has been predicted to result in a phenotype similar to that observed in mucopolysaccharidosis (MPS). In the present study, we surveyed a variety of patients with phenotypes similar to those observed in MPS, but without significant mucopolysacchariduria to determine if some are based on aberrations in serum hyaluronidase (Hyal-1) activity. The study included patients with well-characterized dysmorphic disorders occurring on genetic basis, as well as those of unkown etiology. The purpose of the study was to establish how wide spread were abnormalities in levels of circulating Hyal-1 activity. A simple and sensitive semi-quantitative zymographic procedure was used for the determination of activity. Levels of both beta-N-acetylglucosaminidase and beta-glucuronidase whose activities contribute to the total breakdown of hyaluronan (HA) were also measured, as well as the concentration of circulating HA. Among 48 patients with bone or connective tissue abnormalities, low levels of Hyal-1 activity were found in six patients compared to levels in 100 healthy donors (2.0-3.2 units/microL vs 6(+/- 1 SE) units/microL). These six patients exhibited a wide spectrum of clinical abnormalities, in particular shortened extremities: they included three patients with unknown causes of clinical symptoms, one patient with Sanfilippo disease, one of the seven patients with achondroplasia, and one with hypophosphotemic rickets. Normal levels of serum Hyal-1 activities were found in patients with Morquio disease, GM1 gangliosidosis, I cell-disease, 6 of the 7 patients with achondroplasia, Marfan's-syndrome and Ehlers-Danlos syndrome. No patient totally lacked serum Hyal-1 activity. Serum HA concentration was elevated in patients with Sanfilippo A and I-cell disease. Determination of serum and leukocyte Hyal-1 and serum HA may be useful to evaluate patients with metabolic and morphogenetic disorders.
 ...
PMID:Serum hyaluronidase aberrations in metabolic and morphogenetic disorders. 1631 83