Revisiting human IL-12Rβ1 deficiency: A survey of 141 patients from 30 countries

Interleukin-12 receptor β1 (IL-12Rβ1) deficiency is the most common form of Mendelian susceptibility to mycobacterial disease (MSMD). We undertook an international survey of 141 patients from 102 kindreds in 30 countries. Among 102 probands, the first infection occurred at a mean age of 2.4 years. In 78 patients, this infection was caused by Bacille Calmette-Guérin (BCG; n = 65), environmental mycobacteria (EM; also known as atypical or nontuberculous mycobacteria) (n = 9) or Mycobacterium tuberculosis (n = 4). Twenty-two of the remaining 24 probands initially presented with nontyphoidal, extraintestinal salmonellosis. Twenty of the 29 genetically affected sibs displayed clinical signs (69%); however 8 remained asymptomatic (27%). Nine nongenotyped sibs with symptoms died. Recurrent BCG infection was diagnosed in 15 cases, recurrent EM in 3 cases, recurrent salmonellosis in 22 patients. Ninety of the 132 symptomatic patients had infections with a single microorganism. Multiple infections were diagnosed in 40 cases, with combined mycobacteriosis and salmonellosis in 36 individuals. BCG disease strongly protected against subsequent EM disease (p = 0.00008). Various other infectious diseases occurred, albeit each rarely, yet candidiasis was reported in 33 of the patients (23%). Ninety-nine patients (70%) survived, with a mean age at last follow-up visit of 12.7 years ± 9.8 years (range, 0.5-46.4 yr). IL-12Rβ1 deficiency is characterized by childhood-onset mycobacteriosis and salmonellosis, rare recurrences of mycobacterial disease, and more frequent recurrence of salmonellosis. The condition has higher clinical penetrance, broader susceptibility to infections, and less favorable outcome than previously thought. © 2010 Lippincott Williams & Wilkins.

Schamyl : drame en 5 actes et 9 tableaux. [suivi de] Deux femmes en gage : folie en 1 acte. Livr. 141 / par M. Paul Meurice ; musique de M. Gondoispar MM. Bourdois et Nérée Desarbres

Collection : Théâtre contemporain illustré ; 141e et 142e livraisons

Resolución N° 141 ¿Cuál es el criterio legal sobre el cual se apoya la Cámara de Comercio de Bogotá para evaluar posibles ineficacias de actas y/o documentos sujetos a registro?

Servicios registrales

Copy of page 141 of Confederation by Joseph Pope annotated by John A. Macdonald, 1867

Joseph Pope was born in Charlottetown, Prince Edward Island in 1854. He was the private secretary to Sir John A. Macdonald from 1882-1891. He worked as the assistant clerk to the Privy Council and undersecretary of state for Canada from 1896-1909. He was appointed a Companion of the Order of St. Michael and St. George in 1901. He was later knighted as a Knight Commander of the same order. Joseph Pope was the first permanent head of the Department of External Affairs (now Foreign Affairs and Internal Trade) 1909-1925. He was an advisor to Prime Ministers from Macdonald to King. He died in Ottawa, in 1926. As well as Confederation, Pope also penned: Memoirs of Sir John A. Macdonald : A Chronicle of the First Prime Minister of the Dominion; The Day of Sir John Macdonald; Jacques Cartier, his life and voyages; Traditions and Sir John A. MacDonald vindicated : a review of the Right Honourable Sir Richard Cartwright's reminiscences as well as other books Pope’s son, Maurice Arthur Pope wrote a book about Joseph entitled Public Servant: the Memoirs of Sir Joseph Pope”.

Culture and exopolysaccharide production from sugarcane molasses by Gordonia polyisoprenivorans CCT 7137, isolated from contaminated groundwater in Brazil

Gordonia polyisoprenivorans CCT 7137 was isolated from groundwater contaminated with leachate in an old controlled landfill (Sauo Paulo, Brazil), and cultured in GYM medium at different concentrations of sugarcane molasses (2%, 6%, and 10%). The strain growth was analyzed by monitoring the viable cell counts (c.f.u. mL(-1)) and optical density and EPS production was evaluated at the end of the exponential phase and 24 h after it. The analysis of the viable cell counts showed that the medium that most favored bacterial growth was not the one that favored EPS production. The control medium (GYM) was the one that most favored the strain growth, at the maximum specific growth rate of 0.232 h(-1). Differences in bacterial growth when cultured at three different concentrations of molasses were not observed. Production of EPS, in all culture media used, began during the exponential phase and continued during the growth stationary phase. The highest total EPS production, after 24 h of stationary phase, was observed in 6% molasses medium (172.86 g L(-1)) and 10% (139.47 g L(-1)) and the specific total EPS production was higher in 10% molasses medium (39.03 x10(-11)g c.f.u.(-1)). After the exponential phase, in 2%, 6%, and 10% molasses media, a higher percentage of free exopolysaccharides (EPS) was observed, representing 88.4%, 62.4%, and 64.2% of the total, respectively. A different result was observed in pattern medium, which presented EPS made up of higher percentage of capsular EPS (66.4% of the total). This work is the first study on EPS production by G. polyisoprenivorans strain in GYM medium and in medium utilizing sugarcane molasses as the sole nutrient source and suggests its potential use for EPS production by G. polyisoprenivorans CCT 7137 aiming at application in biotechnological processes.

DOCPAL resúmenes sobre población en América Latina: 141-001

Contiene resúmenes bibliográficos de documentos sobre población y temas relacionados que se producen en América Latina y el Caribe, publicados entre 1987-1989.

O papel das argininas alfa-92 e alfa-141 na regulação das propriedades funcionais de hemoglobinas por íons cloreto

The oxygenation of human Hb (HbA) demands a three state model: two deoxy states To and Tx, free and complexed with anions respectively, and an oxy R state. The regulation between these states is modulated by the presence of anions, such as chloride, that binds to T state. The b inding if chloride, however, remains controversial. The aim of this work is the study of arginines 92a (a1ß2 interface) and 141a (C-terminal) as chloride binding sites. To investigate that, we have studied 92 and 141 site directed mutant species: natural mutants Hb J-Cape-Town (R92Q), desArg (R141Δ), Chesapeake (R92L), and the constructed Chesapeake desArg (R92L,141Δ). We expressed Hbs in Escherichia coli and purified. Through oxygen binding curves we measured affinity and cooperativity, in function of water effect and Bohr effect in presence and absence of chloride. Structural features were obtained through 1H NMR spectroscopy Oxygen binding properties and Bohr effect measured indicated a higher affinity and lower cooperativity in absence and presence of chloride for all mutants. Structural changes represent functional aspects of mutant Hbs, such as a significant rise in affinity or a change in cooperativity. Water activity studies conducted as a function of chloride concentration showed that the only Hb desArg follows the thre state model. The other mutant Hbs do not exhibit the Tx state, a fact confirmed by the number of water molecules bound to each Hb during the deoxy-oxy transition. This behavior suggests that the Arginine 92 site could be responsible for chloride binding to Hb, since oxygenation of 92 mutant Hbs cannot be adjusted by the three state model. However, Bohr effect showed that all mutant Hbs released~1 proton in chloride presence, different from HbA that releases ~2, suggesting a role for 141 arginine in the tertiary and quaternary Bohr effect.

O papel das argininas α-92 e α-141 na regulação da função de hemoglobinas por íons cloreto

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)

Flora polínica da reserva do parque estadual das fontes do Ipiranga (São Paulo, SP, Brasil). Famílias: 141-Boraginaceae e 149-Gesneriaceae

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)