caa a22 4500 465804721 CHVBK 20180323112101.0 cr unu---uuuuu 170327e19901101xx s 000 0 eng 10.1007/BF03349625 doi (NATIONALLICENCE)springer-10.1007/BF03349625 Differential levels of thyroid peroxidase and thyroglobulin messenger ribonucleic acids in congenital goiter with defective thyroglobulin synthesis [Elektronische Daten] [H. Targovnik, V. Varela, G. Juvenal, F. Propato, H. Chester, L. Krawiec, G. Frechtel, D. Moran, H. Perinetti, M. Pisarev] The biosynthesis of thyroid hormones requires iodide, thyroid peroxidase (TPO), thyroglobulin (Tg) and H2O2. We have studied two sisters with congenital large goiters and hypothyroidism. Perchlorate tests were negative. Serum T3 and T4 were decreased, TSH was increased and Tg was within the lower limit of normal. Biochemical and molecular studies were performed on goiter samples obtained after surgery. Tg content in both tissues was negligible. Paper chromatography of labeled iodocompounds showed a decrease in T4, and the presence of a pronase/pan-creatin-resistant iodoprotein. TPO activity was normal in the tissues. Sephacryl S-300 gel filtration demonstrated labeled iodoalbumin-like protein and the absence of a Tg peak. Salting out studies of soluble protein fraction gave an abnormal pattern. Agarose gel electrophoresis showed the presence of an iodoalbumin-like protein and the absence of Tg in the tissues. This last finding was confirmed by immunoelectrophoresis. The Tg and TPO mRNAs levels were also analyzed. Dot-blot hybridization studies with pM5 (TPO cDNA) and phTgM2 (Tg cDNA) probes showed increased and decreased signals, respectively. The increase in TPO mRNA can be explained as a compensatory mechanism vis a vis an increase in serum TSH caused by decreased serum T3 and T4 due to the impairment in Tg mRNA. The Tg mRNA of both patients was further studied with four different probes covering 5′ and 3′ regions (phTgM1, phTgB1, phTgB2 and phTgB3). Hybridization was observed with all four probes, thus excluding a dramatic deletion defect. Northern transfer showed a clear signal of hybridization with the phTgBI probe in the 8-9 Kb range. We may conclude that the biochemical and molecular abnormality of these patients is characterized by a decrease of Tg mRNA and of Tg translation.l higher (p < 0.01) than in normal men. This finding is probably related to the subnormal plasma levels of testosterone found in agonadal men during the replacement therapy; the analysis of data using a sampling interval of 10 min gave results similar to previous reports, confirming that the choice of sampling interval can markedly affect the evaluation of frequent LH pulsatile secretion. Italian Society of Endocrinology (SIE), 1990 Targovnik H. Laboratorio de Biologia Molecular, Hospital de Clinicas José de San Martín, Buenos Aires, Argentina aut Varela V. Laboratorio de Biologia Molecular, Hospital de Clinicas José de San Martín, Buenos Aires, Argentina aut Juvenal G. Divisíon Bioquímica Nuclear, Gerencia de Aplicaciones, Comisión Nacional de Energia Atómica, CNEA, Av del Libertador 8250, 1429, Buenos Aires, Argentina aut Propato F. Laboratorio de Biologia Molecular, Hospital de Clinicas José de San Martín, Buenos Aires, Argentina aut Chester H. Divisíon Bioquímica Nuclear, Gerencia de Aplicaciones, Comisión Nacional de Energia Atómica, CNEA, Av del Libertador 8250, 1429, Buenos Aires, Argentina aut Krawiec L. Divisíon Bioquímica Nuclear, Gerencia de Aplicaciones, Comisión Nacional de Energia Atómica, CNEA, Av del Libertador 8250, 1429, Buenos Aires, Argentina aut Frechtel G. Laboratorio de Biologia Molecular, Hospital de Clinicas José de San Martín, Buenos Aires, Argentina aut Moran D. Centro de Medicina Nuclear, Hospital Central, UNC, Buenos Aires, Argentina aut Perinetti H. Centro de Medicina Nuclear, Hospital Central, UNC, Buenos Aires, Argentina aut Pisarev M. Divisíon Bioquímica Nuclear, Gerencia de Aplicaciones, Comisión Nacional de Energia Atómica, CNEA, Av del Libertador 8250, 1429, Buenos Aires, Argentina aut Journal of Endocrinological Investigation Springer International Publishing 13/10(1990-11-01), 797-806 0391-4097 13:10<797 1990 13 40618 https://doi.org/10.1007/BF03349625 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF03349625 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Targovnik H. Laboratorio de Biologia Molecular, Hospital de Clinicas José de San Martín, Buenos Aires, Argentina aut NATIONALLICENCE 700 1- Varela V. Laboratorio de Biologia Molecular, Hospital de Clinicas José de San Martín, Buenos Aires, Argentina aut NATIONALLICENCE 700 1- Juvenal G. Divisíon Bioquímica Nuclear, Gerencia de Aplicaciones, Comisión Nacional de Energia Atómica, CNEA, Av del Libertador 8250, 1429, Buenos Aires, Argentina aut NATIONALLICENCE 700 1- Propato F. Laboratorio de Biologia Molecular, Hospital de Clinicas José de San Martín, Buenos Aires, Argentina aut NATIONALLICENCE 700 1- Chester H. Divisíon Bioquímica Nuclear, Gerencia de Aplicaciones, Comisión Nacional de Energia Atómica, CNEA, Av del Libertador 8250, 1429, Buenos Aires, Argentina aut NATIONALLICENCE 700 1- Krawiec L. Divisíon Bioquímica Nuclear, Gerencia de Aplicaciones, Comisión Nacional de Energia Atómica, CNEA, Av del Libertador 8250, 1429, Buenos Aires, Argentina aut NATIONALLICENCE 700 1- Frechtel G. Laboratorio de Biologia Molecular, Hospital de Clinicas José de San Martín, Buenos Aires, Argentina aut NATIONALLICENCE 700 1- Moran D. Centro de Medicina Nuclear, Hospital Central, UNC, Buenos Aires, Argentina aut NATIONALLICENCE 700 1- Perinetti H. Centro de Medicina Nuclear, Hospital Central, UNC, Buenos Aires, Argentina aut NATIONALLICENCE 700 1- Pisarev M. Divisíon Bioquímica Nuclear, Gerencia de Aplicaciones, Comisión Nacional de Energia Atómica, CNEA, Av del Libertador 8250, 1429, Buenos Aires, Argentina aut NATIONALLICENCE 773 0- Journal of Endocrinological Investigation Springer International Publishing 13/10(1990-11-01), 797-806 0391-4097 13:10<797 1990 13 40618 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer