Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: KEGG:D02003 (NBT)
 1,323 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 7 year old boy developed in the newborn period a chronic suppurative process after routine BCG vaccination beginning at the site of the injection and spreading to the adjacent areas on neck and chin. A supraclavicular lymphadenopathy was also noted. Serial histological examinations revealed the typical histopathological pattern of tuberculosis and the boy received a tuberculostatic therapy for five years. During this time he suffered from multiple chronic bacterial infections which led to chronic granulomatous inflammations in different organs and to a fibrous pneumonitis with subsequent cor pulmonale. At the age of 6 years a negative NBT-test allowed the diagnosis of GCD. Consequently therapy with Sulfamethoxazol-Trimethoprim was started and the rate of infections diminished markedly.
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PMID:[BCG-infection in chronic granulomatous disease (author's transl)]. 718 85

Chronic granulomatosis disease (GCD) is a hereditary abnormality of phagocytic cells, frequently revealed by invasive pulmonary Aspergillosis. The authors report the case of 8 year old girl investigates for hypotrophia, reccurent lung disease and hemoptysia. She was treated for pulmonary tuberculosis because of biological inflammatory syndrome, right alveolar opacity and positive tuberculin skin test. The course was marked by the developement of a thoracic wall mass. Anti- aspergillus serology was positive; culture of the thoracic mass puncture and bronchoalveolar lavage isolated fumigatus Aspergillus. GCD was suggested and confirmed by the NBT test.
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PMID:[Chronic septic granulomatosis in children revealed by invasive pulmonary aspergillosis]. 1642 74

Two supramolecular complexes, [Cu(L)(H2O)2(beta-CD)](ClO4)2.10.5H2O.CH3OH (1) and [Cu(L)(H2O)2(beta-GCD)](HClO4)(ClO4)2.10H2O (2) (L = 4-(4'-tert-butyl-benzyl)diethylenetriamine, beta-CD = beta-cyclodextrin, and beta-GCD = mono-6-deoxy-6-guanidinocycloheptaamylose cation), have been synthesized. The structure of 1 has been characterized by X-ray crystallography. The 4-tert-butyl-benzyl of [Cu(L)(H2O)2]2+ moiety in 1 as a guest inserts into the hydrophobic cavity of the beta-CD as a host along the primary hydroxyl side. On the basis of the structure data of 1, complex 2 was modeled, which showed that the distance between the Cu and C atom of the guanidinium is 5.2 A, comparable to the corresponding distance in bovine erythrocyte Cu, Zn-SOD (5.9 A) (SOD = superoxide dismutase). Apparent inclusion stability constants of the host and the guest were measured to be 0.66 (+/-0.01) x 104 and 1.15 (+/-0.03) x 104 M-1 for 1 and 2 respectively. The electronic absorption bands and electronic reflection bands of each complex are almost the same, indicating an identical structure of the complex in aqueous solution and in solid state. The two complexes showed quasi-reversible one-electron Cu(II)/Cu(I) redox waves with redox potentials of -0.345 and -0.338 V for 1 and 2, respectively. Their SOD-like activities (IC50) were measured to be 0.30 +/- 0.01 and 0.17 +/- 0.01 microM by xanthine/xanthine oxidase-NBT assay. The enhanced SOD activity of 2 by approximately 40% compared with 1 suggests that the guanidyl cation in the host of the supramolecular system of 2 can effectively mimic the side chain of Arg141 in the enzyme, which is known to be essential for high SOD activity possibly through steering of the superoxide substrate to and from the active copper ion.
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PMID:Synthesis, structure, and activity of supramolecular mimics for the active site and arg141 residue of copper, zinc-superoxide dismutase. 1725 14