Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:1.10.3.1 (tyrosinase)
9,065 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

By cytochemistry (acid phosphatase and tyrosinase activities) GERL, a specialized hydrolase-rich region of endoplasmic reticulum (ER), can be visualized in the cells of the mouse retinal pigment epithelium. Previously catalase cytochemistry permitted us to identify microperoxisomes, with numerous continuities to the ER. The present report reveals the extensive continuities of the ER to pigment granules in various stages of maturation. When the pigment granules, which we consider to be "melanolysosomes," first appear they consist of electron-opaque grains within dilated areas of the ER. As the dilations enlarge, fine fibrils appear in the ER cisternae. Thicker fibers develop from the fibrils; these fibers are generally obscured when melanin deposition occurs. At all stages, the melanolysosomes appear to be connected to the ER.
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PMID:Retinal pigment epithelium. Interrelations of endoplasmic reticulum and melanolysosomes in the black mouse and its beige mutant. 10 66

Chicken embryo retinal pigment cells cultured for 2 weeks in 1 or 10 microns BrdU accumulate only 25% of the amount of melanin found in controls. Growth is inhibited by 10 microns BrdU but not by 1 micron BrdU. Cells cultured with BrdU had decreased tyrosinase activity and lost the typical epithelial appearance of controls. Equimolar concentrations of deoxythymidine did not prevent the inhibition of melanogenesis due to BrdU but did prevent the growth inhibition of the higher concentration of BrdU (10 microns). Tenfold higher concentrations of deoxythymidine prevented inhibition of epithelial differentiation and melanogenesis. Ultrastructurally, BrdU produced an absence of melanosomes and disruption of the lamellar array of rough endoplasmic reticulum. The perinuclear arrangement of microfilaments became irregular. The cell surface was radically affected as membranes were no longer ruffled and cell junctions disappeared. Melanogenesis is a complex form of differentiated function, involving synthesis of tyrosinase and control of its activity, macromolecular synthesis of the melanosome, and the subsequent synthesis of melanin within the melanosome. BrdU appears to inhibit melanogenesis through a coordinated and simultaneous interference with these processes, suggesting that there may be a "program" of gene activity for melanogenesis that is regulated as a unit.
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PMID:Effect of 5-bromo-2'-deoxyuridine on growth and differentiation of cultured embryonic retinal pigment cells. 11 5

Conley et al., in 1971, described a special type of melanoma characterized by a superficial melanic lesion at the onset; repeated local relapses as subcutaneous tumorations with an histological picture closely resembling an atypical fibroxantoma or fibrosarcoma. After a review of all the published material the autors presents a personal case with the clinical, histological and evolutive characteristics of this disease. The most interesting findings of the published case are the following: The special stains for the melanocytes (silver stain, Dopa, tyrosinase and cholinesterase) were all negative. There was an intense positivity for the lisosomal enzymes (non specific sterases, and acid phosphatases). The ultrastructural study of the tumoral tissues as well as the cells of cultures showed abundant cells with tumoral aspects, with prominent nucleoli somewhat dilated granular endoplasmic reticulum, myelin-like figures, lipidic vacuoles and abundant lisosomes. No melanosomes or premelanosomes were observed. Beside these tumoral cells abundant typical fibroblastic elements were found. There was a great amount of collagen fibers with periodicity superior to the normal. The conclusion is that the desmoplastic melanoma must be considered as a tumor of mesenchimatous origin intervening in its development multiple local and general factors.
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PMID:[Desmoplastic melanoma]. 34 19

Human malignant melanocytes show characteristic morphologic modifications which are particularly evident in their specific organelles: melanosomes. These modifications are conserved in cell culture. The ultrastructural localization of tyrosinase, the enzyme which converts tyrosine and dopa into melanin, was determined in 13 human melanoma cell lines. The different cell lines possess 4 distribution patterns of melanin synthesis based on dopa oxidase activity. The two first pathways, which involve the Golgi apparatus, seem to differ by the amount of enzyme within this organelle. The third pathway mainly involves the smooth endoplasmic reticulum, whereas tyrosinase is visible only in vesicles in the fourth. Some cells synthesize the enzyme in the manner observed in very early embryos.
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PMID:Cellular localization of tyrosinase in human malignant melanoma cell lines. 40 30

Ultrastructural studies, and cytochemical and biochemical determinations of tyrosinase activity were conducted on the pigment epithelium of albino and xanthic goldfish eyes. In eyes of xanthic goldfish, two types of melanosomes are present, spherical and elongated. Melanized melanosomes are absent in the eyes of the albino goldfish, but elongated lamellar premelanosomes are observed. Internal vesicles are present in both melanosome types in the pigment epithelium of the xanthic goldfish but are absent in premelanosomes of the albino. There are also differences in the distribution of lipid droplets, smooth endoplasmic reticulum and Golgi complexes with the latter two being more abundant in the albino. Tyrosinase was not identified cytochemically; however, the enzyme was demonstrated biochemically in the pigment epithelia of both albino and xanthic goldfish. The enzyme is associated with the particulate and soluble fractions fo both types of eyes. Particulate albino tyrosinase may be solubilized by triton X-100 treatment. Tyrosinase inhibitors are present in the particulate fractions of both albino and xanthic goldfish eyes. Thus, in the goldfish, ocular albinism appears to be a multiple defect at the molecular and ultrastructural levels.
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PMID:Trysinase positive oculocutaneous albinism in the goldfish, Carassius auratus l., and ultrastructural and biochemical study of the eye. 41 72

The eyes of a 13-year-old leukemic boy with the attributes of tyrosinase-negative oculocutaneous albinism were obtained for light and electron microscopic study. Repeated examinations had failed to reveal WBCs with giant oxidase-positive granules, and leukemic involvement of the fundus never occurred. Light microscopic examination of horizontal and vertical sections through the retina confirms earlier reports that the fovea is absent in albinos. The synaptic apparatus of the photoreceptor terminals appears abnormal. The rough endoplasmic reticulum of the retinal pigment epithelial cells is sparse though the presence of phagosomes suggests that phagocytic function is intact. Suggestions as to the importance that the morphological findings may have on albino visual function are made.
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PMID:Human albinism. Light and electron microscopy study. 62 78

Two enzymes with tyrosinase activity have been purified from the frog Rana pipiens. Both enzymes are isolated in an inactive form which can be activated with trypsin. Amino acid analysis, NH2-terminal amino acid determination (arginine for both proteins), and immunological evidence indicate that athe two enzymes are similar if not identical. They can be distinguished by their trypsin activation kinetics. Cell fractionation studies suggest that one form is found associated with the smooth endoplasmic reticulum whereas the other protein fraction is localized mainly within the premelanosomes. Sedimentation equilibrium studies demonstrate that both protein fractions are self-associating systmes. Ionic strength, temperature, and specific anion effects alter the equilibria of the associating systems. The monomeric molecule weight for both fractions is 30,000 and at low ionic strengths the predominant molecular weight species is the tetramer. The partial specific volume of each protein is 0.70.
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PMID:Tyrosinases in Rana pipiens. Purification and physical properties. 80 96

The iris anlage of 2-10 and 15 days old chicken embryos were studied histochemically, and by both light and electron microscopy. Light microscopic serial sections showed that pigmentation began at the outer layer of the posterior eye pole and progressed from there forwards to the optic cup margin. The entire outer layer of the optic cup as well as the pupillary margin were completely pigmented by the 4th day of incubation. By the 10th day the posterior iris epithelium was totally pigmented. Electron microscopical studies showed that the first premelanosomes appeared at about the 3rd day of incubation in the anterior iris anlage with the exception of the pupillary margin. It could be shown that melanogenesis progressed through the following steps: premelanosomes, followed by tyrosinase activity in a Golgi-associated system of smooth endoplasmic reticulum (GERL) and small vesicles and finally differentiation of the melanosomes. The possible origin of the premelanosomes and the formation of melanin are discussed.
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PMID:Melanogenesis in the pigment epithelium of chicken embryos. I. Topogenesis of pigment in the iris anlage. 81 42

Tyrosinase is synthesized on membrane-bound ribosomes and transported into melanosomes through smooth endoplasmic reticulum and Golgi apparatus. Melanin polymers are produced only in melanosomes but never in smooth endoplasmic reticulum or Golgi apparatus, indicating that posttranslational modifications of tyrosinase are completed with melanosomes where tyrosinase becomes an active form. Based on a working hypothesis that tyrosinase-positive oculocutaneous albinism is a consequence of the structurally altered tyrosinase due to a point mutation in the gene of its gene coding for a glycosylation site or a membrane-binding site, which leads to the impairment in the posttranslational modification of tyrosinase and its catalytic activity, we have cloned the tyrosinase gene of one patient affected with tyrosinase-positive oculocutaneous albinism and determined its nucleotide sequence. Thus demonstrated all exons' nucleotide sequence of the patient's tyrosinase gene was found to be identical to that of the wild-type gene. The results indicate that the patient's tyrosinase itself is not altered. We therefore propose that the molecular basis for the development of tyrosinase-positive oculocutaneous albinism exists as a defect in other proteins required for the activation of tyrosinase or in other regions of the tyrosinase gene.
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PMID:Cloning and sequence analysis of the tyrosinase gene from a patient with tyrosinase-positive oculocutaneous albinism. 149 98

Electron micrographs of skin from xanthic (gold) sailfin mollies revealed numerous xanthophores, as well as scattered melanophores. The melanophores were seen to contain premelanosomes in various stages of development. This is consistent with the fact that xanthic mollies have been shown to be tyrosinase positive. Melanosomes in xanthic mollies appear to develop by one of two pathways: 1) from an endoplasmic reticulum-derived vesicle which develops an internal lamellar framework, and 2) by fusion of multiple Golgi-derived vesicles which lack an internal lamellar framework. Analysis of the pigments in the skin of the xanthic mollies identified four colorless pteridine pigments (xanthopterin, isoxanthopterin, neopterin, and pterin) and a carotenoid with an absorbance spectrum similar to beta-carotene. It appears that, unlike some other poeciliid fishes, sailfin mollies do not use pteridine pigments for orange coloration. Rather, they appear to rely primarily on carotenoids.
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PMID:Pigments and ultrastructures of pigment cells in xanthic sailfin mollies (Poecilia latipinna). 182 28


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