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Abstract Mucolipidosis II and III (MLII and MLIII) are autosomal recessive diseases caused by pathogenic variants in GNPTAB and GNPTG genes that lead to defects in GlcNAc-1-phosphotransferase. This enzyme adds mannose 6-phosphate residues to lysosomal hydrolases, which allows enzymes to enter lysosomes. Defective GlcNAc-1-phosphotransferase causes substrate accumulation and inflammation. These diseases have no treatment, and we hypothesized that the use of substrate reduction therapy and immunomodulation may be beneficial at the cell level and as a future therapeutic approach. Fibroblasts from two patients with MLIII alpha/beta and 2 patients with MLIII gamma as well as from one healthy control were treated with 10 µM miglustat, 20 µM genistein, and 20 µM thalidomide independently. ELISA assay and confocal immunofluorescence microscopy were used to evaluate the presence of heparan sulfate (HS) and the impact on substrate accumulation. ELISA assay showed HS reduction in all patients with the different treatments used (p=0.05). HS reduction was also observed by immunofluorescence microscopy. Our study produced encouraging results, since the reduction in substrate accumulation, even partial, may offer benefits to the phenotype of patients with inborn errors of metabolism.

https://doi.org/10.1590/1678-4685-gmb-2023-0117

It is not just a field on a form: maternity on the Sucupira Platform form
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STANISCUASKI, FERNANDA

; ZANDONA, EUGENIA

; REICHERT, FERNANDA

; SOLETTI, ROSSANA C.

; MELLO-CARPES, PAMELA B.

; WIGGERS, GIULIA A.

; SCHWARTZ, IDA V.D.

; WERNECK, FERNANDA P.

; LUDWIG, ZELIA M.C

; NEUMANN, ADRIANA

; ALMEIDA, CAMILA INFANGER

; OLIVEIRA, LETICIA DE

Anais da Academia Brasileira de Ciências

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2023, Volume 95 N. 2 elocation e20230135

Abstract: En | Text: En | PDF: En

https://doi.org/10.1590/0001-3765202320230135

Characterization of the 3'UTR of the BTD gene and identification of regulatory elements and microRNAs
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Silva, Gerda Cristal Villalba

; Borsatto, Taciane

; Schwartz, Ida Vanessa Doederlein

; Sperb-Ludwig, Fernanda

Genetics and Molecular Biology

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2022, Volume 45 N. 1 elocation e20200432

Abstract: En | Text: En | PDF: En

Abstract Reduced biotinidase activity is associated with a spectrum of deficiency ranging from total deficiency to heterozygous levels, a finding that is not always explained by the pathogenic variants observed in the BTD gene. The investigation of miRNAs, regulatory elements and variants in the 3’UTR region may present relevance in understanding the genotype-phenotype association. The aims of the study were to characterize the regulatory elements of the 3’UTR of the BTD gene and identify variants and miRNAs which may explain the discrepancies observed between genotype and biochemical phenotype. We evaluated 92 individuals with reduced biotinidase activity (level of heterozygotes = 33, borderline = 35, partial DB = 20 or total DB= 4) with previously determined BTD genotype. The 3’UTR of the BTD gene was Sanger sequenced. In silico analysis was performed to identify miRNAs and regulatory elements. No variants were found in the 3’UTR. We found 97 possible miRNAs associated with the BTD gene, 49 predicted miRNAs involved in the alanine, biotin, citrate and pyruvate metabolic pathways and 5 genes involved in biotin metabolism. Six AU-rich elements were found. Our data suggest variants in the 3'UTR of BTD do not explain the genotype-phenotype discrepancies found in Brazilian individuals with reduced biotinidase.

https://doi.org/10.1590/1678-4685-gmb-2020-0432

A decade of molecular diagnosis of Mucolipidosis II and III in Brazil: a pooled analysis of 32 patients
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Ludwig, Nataniel F

; Sperb-Ludwig, Fernanda

; Randon, Dévora N

; Bernardi, Pricila

; Giuliani, Liane R

; Moreno, Carolina A

; Cavalcanti, Denise P

; Silva, Luiz CS da

; Schwartz, Ida V D

Journal of Inborn Errors of Metabolism and Screening

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2021, Volume 9 elocation e20200029

Abstract: En | Text: En | PDF: En

Abstract GlcNAc-1-phosphotransferase is a hexameric complex formed by subunits α, β, and γ, where the first two are encoded by the GNPTAB gene and the third by the GNPTG gene. Pathogenic variants identified in the GNPTAB gene cause the diseases Mucolipidosis II and III alpha/beta, which are severe and characterized by an overflow of lysosomal hydrolases into the extracellular environment, and their absence in lysosomal compartments causes an accumulation of non-degraded macromolecules. Methodology: a retrospective study that included 32 unrelated Brazilian patients with a clinical and genetic diagnosis of Mucolipidosis II/III alpha/beta. The regional frequency of the altered alleles was determined. Results: The patients were from all regions of Brazil. The most prevalent variants were c.3503_3504del, associated with the severe form of the disease, and c.1208T>C, associated with the milder form. Variant c.3503_3504del is the most frequently found in the Midwest, Northeast, and Southeast regions of Brazil. In the South, 42.8% of the alleles present the c.1196C>T variant. Conclusions: From the perspective of all patients diagnosed with Mucolipidosis II/III in Brazil, it is possible to conclude that different regions present allelic frequencies of specific pathogenic variants, which can be explained by the occurrence of a founding effect or high inbreeding rates.

https://doi.org/10.1590/2326-4594-jiems-2020-0029 225 downloads

Maternity in the Brazilian CV Lattes: when will it become a reality?
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STANISCUASKI, FERNANDA

; ZANDONÀ, EUGENIA

; REICHERT, FERNANDA

; SOLETTI, ROSSANA C.

; OLIVEIRA, LETICIA DE

; RICACHENEVSKY, FELIPE K.

; TAMAJUSUKU, ALESSANDRA S.K.

; KMETZSCH, LIVIA

; SCHWARTZ, IDA V.D.

; WERNECK, FERNANDA P.

; LUDWIG, ZELIA M.C

; LIMA, ELIADE F.

; INFANGER, CAMILA

; NEUMANN, ADRIANA

; BRANDÃO, ALESSANDRA

; WIGGERS, GIULIA A.

; SEIXAS, ADRIANA

; MELLO-CARPES, PAMELA B.

Anais da Academia Brasileira de Ciências

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2021, Volume 93 N. 1 elocation e20201370

Abstract: En | Text: En | PDF: En

https://doi.org/10.1590/0001-3765202120201370 1955 downloads

The fructose-1,6-bisphosphatase deficiency and the p.(Lys204ArgfsTer72) variant
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Pinheiro, Franciele Cabral

; Ligabue-Braun, Rodrigo

; Siqueira, Ana Cecília Menezes de

; Matuella, Camila

; Souza, Carolina Fischinger Moura de

; Monteiro, Fabíola Paoli

; Kok, Fernando

; Schwartz, Ida Vanessa Doederlein

; Sperb-Ludwig, Fernanda

Genetics and Molecular Biology

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2021, Volume 44 N. 2 elocation e20200281

Abstract: En | Text: En | PDF: En

Abstract Fructose-1,6-bisphosphatase (FBPase) deficiency is a rare inborn error of fructose metabolism caused by pathogenic variants in the FBP1 gene. As gluconeogenesis is affected, catabolic episodes can induce ketotic hypoglycemia in patients. FBP1 analysis is the most commonly used approach for the diagnosis of this disorder. Herein, a Brazilian patient is reported. The proband, a girl born to a consanguineous couple, presented with severe hypoglycemia crisis in the neonatal period. At the age 17 months, presented a new crisis accompanied by metabolic acidosis associated with a feverish episode. Genetic analysis was performed by next-generation sequencing (NGS), identifying the NM_000507.3:c.611_614del variant in homozygosis in the FBP1 gene. In silico analysis and 3D modeling were performed, suggesting that this variant is associated with a loss of sites for substrate and Mg2+ binding and for posttranslational modifications of FBPase. The c.611_614del variant is located in a repetitive region of the FBP1 gene that appears to be a hotspot for mutational events. This frameshift creates a premature termination codon in the last coding exon which escapes the nonsense-mediated decay mechanism, according to in silico analysis. This variant results in an intrinsically disordered protein with loss of substrate recognition and post-translational modification sites.

https://doi.org/10.1590/1678-4685-gmb-2020-0281 308 downloads

Molecular detection of Apicomplexa protozoa in tissues from Alouatta guariba clamitans
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Ludwig, Aline

; Murer, Laurete

; Santos, Helton F. dos

; Ludwig, Adriana

; Sangioni, Luis Antonio

; Vogel, Fernanda S.F.

Pesquisa Veterinária Brasileira

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2021, Volume 41 elocation e06717

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

RESUMO: O bugio ruivo (Alouatta guariba clamitans) é uma espécie de primata amplamente distribuída na América do Sul. As infecções por protozoários são comuns em primatas. Entretanto, estudos sobre protozoários em primatas no Brasil são escassos, portanto o objetivo deste estudo foi pesquisar DNA dos protozoários Apicomplexa Neospora caninum, Sarcocystisspp. e Toxoplasma gondii em tecidos de A. guariba clamitans. A extração de DNA foi realizada em amostras de tecido do coração, cérebro, fígado, baço, pulmão e intestino de seis A. guariba clamitans oriundos de Santa Maria, Região Central do Rio Grande do Sul, Brasil. Foi realizada PCR convencional utilizando primers geral do gene 18S rRNA para Apicomplexa e também primers específicos para amplificação de DNA de Neospora spp.e Toxoplasma gondii. Todos os animais foram positivos no PCR geral para Apicomplexa e no sequenciamento genético confirmou-se a presença de DNA de Sarcocystis nos tecidos de quatro animais pertencentes a pelo menos duas espécies (S. neurona e S. gigantea), e DNA de T. gondii foi detectado nos outros dois animais. Uma amostra positiva para T. gondii foi caracterizada genotipicamente como atípico pela técnica de polimorfismo do comprimento do fragmento de restrição. Não foi detectado DNA de N. caninum nas amostras testadas. A presença de DNA de protozoários apicomplexa nos tecidos dos seis animais testados neste estudo destaca a importância dos bugios ruivos como mantenedores desses patógenos na natureza.

ABSTRACT: The brown howler monkey (Alouatta guariba clamitans) is a primate species widely distributed in South America. Infections by protozoa are common in primates. However, studies on protozoa in primates in Brazil are scarce, so the goal of this study was to investigate DNA from the apicomplexan protozoa Neospora caninum, Sarcocystis spp. and Toxoplasma gondii in tissues of A. guariba clamitans. DNA extraction was performed on tissue samples from the heart, brain, liver, spleen, lung and intestine of six A. guariba clamitans from Santa Maria, Central Region of Rio Grande do Sul, Brazil. Conventional PCR was performed using 18S rRNA gene general primers for Apicomplexa and also specific primers to amplify Neosporaspp. and Toxoplasma gondii DNA. All animals were positive in the 18S PCR and the genetic sequencing confirmed the presence of Sarcocystis spp. DNA in the tissues of four animals belonging to at least two species (S. neurona and S. gigantea) and T. gondii DNA in the other two animals. One positive sample for T. gondii was genotypically characterized as atypical by the restriction fragment length polymorphism technique. N. caninum DNA was not detected in the tested samples. The presence of Apicomplexa protozoan DNA in the tissues of the six animals tested in this study highlights the importance of howler monkeys as maintainers of these pathogens in nature.

https://doi.org/10.1590/1678-5150-pvb-6717 207 downloads

COVID-19 Pandemic: health professionals’ perception about the assistance mentioned in television media
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Ludwig, Erika Fernanda dos Santos Bezerra

; Fracasso, Nathalia Vasconcelos

; Faggion, Renata Pires de Arruda

; Silva, Stephanye Vithória Martins da

; Silva, Larissa Gutierrez de Carvalho

; Haddad, Maria do Carmo Fernandez Lourenço

Revista Brasileira de Enfermagem

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2021, Volume 74 Suppl. 1 elocation e20201258

Abstract: Pt En Es | Text: Pt En Es | PDF: Pt | PDF: En | PDF: Es

RESUMO Objetivo: Desvelar a percepção dos profissionais de saúde sobre a assistência prestada durante a pandemia da COVID-19, aludida em mídia televisiva. Métodos: Estudo qualitativo desenvolvido após análise de vídeos com depoimentos de profissionais atuantes na assistência a pacientes com COVID-19, apresentados pelo quadro “Aqui dentro”, do Jornal Nacional da Rede Globo de Televisão, transmitidos em 2020. Os dados textuais foram processados pelo software IRAMUTEQ com classificação hierárquica descendente e análise de conteúdo, sendo o referencial teórico, a psicodinâmica do trabalho de Dejours. Resultados: Emergiram três categorias: Sobrecarga de trabalho na prestação da assistência; Mobilização subjetiva dos profissionais de saúde; Estratégias para enfrentamento na prestação da assistência. Considerações finais: Verificou-se a sobrecarga de trabalho físico e psíquico durante a assistência, mas com preocupação em garantir cuidado humanizado. Apesar das dificuldades enfrentadas, a recuperação e alta dos pacientes geraram motivação e satisfação diante do cenário da COVID-19.

ABSTRACT Objective: Exhibit the health professionals’ perception about the assistance provided during the COVID-19 pandemic mentioned television media. Methods: Qualitative study developed after analysis of the videos with a testimonial from practitioners assisting patients with COVID-19, presented by the series “Inside Here” (in Portuguese, “Aqui Dentro”), of Globo TV News Jornal Nacional, broadcasted in 2020. Textual data was processed by the software IRAMUTEQ with descending hierarchical classification and content analysis, having the theoretical framework as the psychodynamics of Dejours’ study. Results: Three categories emerged: work overload in assisting; subjective mobilization of health professionals; strategies to face in assisting. Final considerations: We verified the physical and psychic work overload during the assistance, but there was a concern to ensure a humanized care. Despite the difficulties faced, the recovery and discharge from patients generated motivation and satisfaction in front of the COVID-19 scenario.

RESUMEN Objetivo: Desvelar la percepción de profesionales de salud sobre la asistencia prestada durante la pandemia de COVID-19, aludida en medio televisivo. Métodos: Estudio cualitativo desarrollado después de análisis de vídeos con deposiciones de profesionales actuantes en la asistencia a pacientes con COVID-19, presentados por el cuadro “Aquí dentro”, del Periódico Nacional de la Rede Globo de Televisión, transmitidos en 2020. Datos textuales fueron procesados por software IRAMUTEQ con clasificación jerárquica descendente y análisis de contenido, siendo el referencial teórico, la psicodinámica del trabajo de Dejours. Resultados: Emergieron tres categorías: Sobrecarga de trabajo en prestación de asistencia; Movilización subjetiva de profesionales de salud; Estrategias para enfrentamiento en prestación de asistencia. Consideraciones finales: Verificó la sobrecarga de trabajo físico y psíquico durante la asistencia, pero con preocupación en garantizar cuidado humanizado. Aunque las dificultades enfrentadas, la recuperación y alta de los pacientes generaron motivación y satisfacción frente al escenario de COVID19.

https://doi.org/10.1590/0034-7167-2020-1258 484 downloads

Extremely low-oxygen storage: aerobic, anaerobic metabolism and overall quality of apples at two temperatures
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Ludwig, Vagner

; Thewes, Fabio Rodrigo

; Wendt, Lucas Mallmann

; Berghetti, Magno Roberto Pasquetti

; Schultz, Erani Eliseu

; Schmidt, Suele Fernanda Prediger

; Brackmann, Auri

Bragantia

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sep 2020, Volume 79 N. 3 Pages 458 - 471

Abstract: En | Text: En | PDF: En

ABSTRACT The objective of this work was to evaluate the effect of extremely low-oxygen storage on the aerobic, anaerobic metabolism and overall quality maintenance of ‘Royal Gala’ and ‘Galaxy’ apples stored at two temperatures. For ‘Royal Gala’ apples, four treatments were evaluated in a two-factor scheme (2 × 2), with two storage conditions: [1] 1.2 kPa O2 + 2 kPa CO2; [2] 0.8 kPa O2 + 1.6 kPa CO2; and two temperatures (1.0 and 1.5 °C). For ‘Galaxy’ apples, the experiment was conducted in a two-factor (3 × 2) scheme with three storage conditions: [1] 1.2 kPa O2 + 2 kPa CO2; [2] 0.8 kPa O2 + 1.6 kPa CO2; [3] 0.4 kPa O2 + 1.2 kPa CO2, and two temperatures (1.0 and 1.5 °C). The ‘Royal Gala’ apples stored under 0.8 kPa O2 + 1.6 kPa CO2 at 1.5 °C had higher flesh firmness, healthy fruit and soluble solids along with lower incidence of flesh breakdown and mealiness. The ‘Galaxy’ apples stored under 0.4 kPa O2 + 1.2 kPa CO2, at temperature of 1.5 °C had lower 1-carboxylic acid-1-aminocyclopropane (ACC) oxidase enzyme activity, ethylene production, occurrence of mealiness, when compared to 1.0 °C. The storage of these two cultivars at 1.5 °C with extremely low O2 partial pressures is possible. The possibility to increase the storage temperature from 1 °C, which is traditionally used for these mutants, to 1.5 °C allows to keep better quality with the advantage of energy saving.

https://doi.org/10.1590/1678-4499.20190496 561 downloads

10.

Extremely low oxygen partial pressures for the storage of ‘Royal Gala’ apple
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Berghetti, Magno Roberto Pasquetti

; Brackmann, Auri

; Thewes, Fabio Rodrigo

; Schultz, Erani Eliseu

; Ludwig, Vagner

; Wendt, Lucas Mallmann

; Thewes, Flavio Roberto

; Schmidt, Suele Fernanda Prediger

Bragantia

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mar 2020, Volume 79 N. 1 Pages 145 - 154

Abstract: En | Text: En | PDF: En

ABSTRACT The aim of this study was to evaluate the effect of storing ‘Royal Gala’ apple under extremely low oxygen partial pressures on their anaerobic metabolism, ethylene production and fruit quality after 9 months of storage plus 7 days of shelf life at 20 °C. The storage conditions tested were: 1.2 kPa O2 + 2.0 kPa CO2; 0.7 kPa O2 + 1.5 kPa CO2; 0.5 kPa O2 + 1.2 kPa CO2; 0.4 kPa O2 +1.2 kPa CO2 and 0.25 kPa O2 + 0.0 kPa CO2. The fruit storage temperature was 1.0 °C (± 0.1). Fruit stored at 0.4 kPa O2 + 1.2 kPa CO2 maintained higher flesh firmness and a higher percentage of healthy fruit. The fruit stored under the 0.25 kPa O2 + 0.0 kPa CO2 condition had lower flesh firmness as well as a reduced percentage of healthy fruit and a high production of anaerobic metabolism compounds, such as ethanol and ethyl acetate, which may have caused fruit quality loss. The storage of ‘Royal Gala’ apple under 0.4 kPa O2 + 1.2 kPa CO2 is a technique that can be commercially applied to keep a better fruit quality after a 9 months storage period.

https://doi.org/10.1590/1678-4499.20190016 644 downloads

11.

Prevalence of the most common pathogenic variants in three genes for inborn errors of metabolism associated with sudden unexpected death in infancy: a population-based study in south Brazil
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Randon, Dévora N.

; Sperb-Ludwig, Fernanda

; Vianna, Fernanda S. L.

; Becker, Ana P. P.

; Vargas, Carmen R.

; Sitta, Angela

; Sant’Ana, Alexia N.

; Schwartz, Ida V. D.

; Bitencourt, Fernanda H. de

Genetics and Molecular Biology

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2020, Volume 43 N. 3 elocation 20190298

Abstract: En | Text: En | PDF: En

Abstract Citrullinemia type 1 (CTLNI), long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD), and mut0 methylmalonic acidemia (mut0 MMA) are inborn errors of metabolism (IEMs) associated with sudden unexpected death in infancy (SUDI). Its most common pathogenic variants are: c.1168G>A (CTLNI, ASS1 gene), c.1528G>C (LCHADD, HADHA gene), c.655A>T and c.1106G>A (mut0 MMA, MUT gene). Considering the absence of estimates regarding the incidence of these diseases in Brazil, this study sought to investigate the prevalence of its main pathogenic variants in a healthy population in the southern region of the country. A total of 1,000 healthy subjects from Rio Grande do Sul were included. Genotyping was performed by real-time PCR. Individuals found to be heterozygous for c.1528G>C underwent further acylcarnitine profile analysis by tandem mass spectrophotometry. Allele and genotype frequencies were calculated considering Hardy-Weinberg equilibrium. The c.1528G>C variant was detected in heterozygosity in two subjects (carrier frequency = 1:500; allele frequency = 0.001; minimum prevalence of LCHADD = 1: 1,000,000), whose acylcarnitine profiles were normal. Variants c.1168G>A, c.655A>T, and c.1106G>A were not identified. These results denote the rarity of these IEMs in Southern Brazil, highlighting the need to expand the investigation of IEMs in relation to infant morbidity and mortality within the country.

https://doi.org/10.1590/1678-4685-gmb-2019-0298 803 downloads

12.

Methods of potassium contents evaluation in the substrate solution and gerbera leaves
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Ludwig, Fernanda

; Fernandes, Dirceu Maximino

; Guerrero, Amaralina Celoto

; Ferreira, Guilherme Amaral

; Pohlmann, Valeria

Ornamental Horticulture

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sep 2019, Volume 25 N. 3 Pages 238 - 246

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

RESUMO Vários autores relatam que o potássio (K) é o nutriente absorvido em maiores quantidades pelas plantas de gérbera. Assim, objetivou determinar a concentração de K na solução de substratos no cultivo de gérbera de vaso, quantificado pelo Cardy Horiba C-131 e espectrofotometria de absorção atômica e correlacioná-los entre si e com o teor foliar na planta. O experimento foi realizado em casa de vegetação. O delineamento experimental utilizado foi o de blocos ao acaso em esquema fatorial 5×2 (5 substratos/2 cultivares) e quatro repetições. As mudas de gérbera cultivares Cherry e Red, com quatro folhas definitivas foram transplantadas em vasos preenchidos com os substratos e aclimatadas durante 30 dias. Após a aclimatação, avaliou-se quinzenalmente o teor de K na solução do substrato extraída pela metodologia do “PourThru”, e o teor foliar de potássio das plantas de gérbera, utilizando-se o medidor de íons portátil Cardy Horiba C-131 e o espectrofotômetro de absorção atômica. A solução do substrato comercial apresentou maiores concentrações de K em relação aos demais nas duas formas de medição, o que está relacionado diretamente com os elevados teores iniciais do nutriente nesse substrato. Valores maiores de K foram obtidos para a cultivar Red, tanto na solução quanto nas folhas. A concentração de K da solução do substrato cultivado com plantas de gérbera envasada quantificada pelo Cardy Horiba C-131 e pela espectrofotometria de absorção atômica correlacionaram-se significativamente entre si, e apresentaram baixa correlação com o teor desse nutriente nas folhas.

Abstract Several authors report that potassium (K) is the nutrient absorbed in greater amounts by gerbera plants. Thus, objective of determining the concentration of Kin the solution of substrates for growing potted gerbera, quantified by the Cardy Horiba C-131 and an atomic absorption spectrophotometer, correlating them with each other and with the plant leaf content. The experiment was carried out in a greenhouse. The experimental design was in randomized complete blocks using a 5×2 factorial scheme (5 substrates/2 cultivars) and four replications. Cherry and Red gerbera cultivar seedlings with four final leaves were transplanted into pots, filled with the substrates and acclimated for 30 days. After acclimation, the K content in the substrate solution extracted by the “PourThru” methodology was evaluated every two weeks using the Cardy Horiba C-131 portable ion meter and an atomic absorption spectrophotometer, as well as the K content in the leaves of gerbera plants. The solution of the commercial substrate had higher concentrations of K in relation to the others in the two forms of measurement, which is directly related to the high initial contents of the nutrient in this substrate. This is directly related to the high initial K contents in the substrate. Greater values of K were obtained for the cultivar Red, both in the solution and in the leaves. The K concentration of the substrate solution cultured with gerbera plants quantified by the Cardy Horiba C-131 and atomic absorption spectrophotometry correlated significantly with each other and showed a low correlation with the content of this nutrient in the plant tissue.

https://doi.org/10.1590/2447-536x.v25i3.1535 1254 downloads

13.

Humoral immune response in adult Brazilian patients with Mucolipidosis III gamma
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Sperb-Ludwig, Fernanda

; Alegra, Taciane

; Velho, Renata Voltolini

; Ludwig, Nataniel

; Siebert, Marina

; Jobim, Mariana

; Vairo, Filippo

; Schwartz, Ida V. D.

Genetics and Molecular Biology

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sep 2019, Volume 42 N. 3 Pages 571 - 573

Abstract: En | Text: En | PDF: En

Abstract Mucolipidosis II and III (ML II and III) alpha/beta and ML III gamma are lysosomal diseases caused by GlcNAc-1-phosphotransferase deficiency. Previous data indicate that MLII patients have functionally impaired immune system that contributes to predisposition to infections.We evaluated the immunological phenotype of three Brazilian patients with ML III gamma. Our data suggest that the residual activity of GlcNAc-1-phosphotransferase in patients with ML III gamma is enough to allow the targeting of the lysosomal enzymes required for B-cell functions maintenance.

https://doi.org/10.1590/1678-4685-gmb-2018-0246 589 downloads

14.

Development and quality of gladiolus stems with the use of vermicompost and Trichoderma sp. in substrate (1)
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Cruz, Letícia Rutz Dewantier da

; Ludwig, Fernanda

; Steffen, Gerusa Pauli Kist

; Maldaner, Joseila

Ornamental Horticulture

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mar 2018, Volume 24 N. 1 Pages 70 - 77

Abstract: Pt En | Text: Pt En | PDF: Pt | PDF: En

RESUMO O gladíolo é uma importante flor de corte cultivada em todo o mundo, muito apreciada para uso em arranjos florais. A crescente preocupação com a contaminação ambiental e a busca pela produção com maior qualidade, tem resultado no aumento dos estudos com o uso de insumos orgânicos e microrganismos na floricultura, destacando-se o vermicomposto e o Trichoderma sp. O presente trabalho foi conduzido com o objetivo avaliar o desenvolvimento e a qualidade de hastes florais de gladíolo em função do uso de vermicomposto e de Trichoderma sp. aplicados ao substrato. O experimento foi conduzido em casa de vegetação, de agosto a dezembro de 2015. Foram utilizados quatro tratamentos: T1 = substrato controle (50% solo e 50% substrato comercial Carolina Soil®, T2 = vermicomposto (40%) e substrato controle (60%); T3 = Trichoderma sp. adicionado ao substrato controle; T4 = Trichoderma sp. adicionado ao vermicomposto (40%) e substrato controle (60%). A variedade de gladíolo utilizada foi a Peter's Pears. As variáveis analisadas foram: altura da planta principal, índice de clorofila Falcker, comprimento e diâmetro da haste principal, comprimento do pendão, número de floretes da haste principal, proporcionalidade entre pendão e haste e tempo até o início da floração. Concluiu-se que o desenvolvimento e a qualidade das hastes florais de gladíolo foram favorecidos pelo uso do vermicomposto na concentração de 40%, adicionado ao substrato.

ABSTRACT The gladiolus is an important cut flower that is cultivated in all parts of the world, and it is very appreciated to be used in floral arrangements. The growing concern with the environmental contamination and the look for production with higher quality have resulted in increasing the number of studies on the use of organic inputs and microorganisms in floriculture, in particular, the vermicompost and the Trichoderma sp. This work was conducted with the objective to evaluate the development and the quality of gladiolus floral stems in function of the use of vermicompost and Trichoderma sp. applied to the substrate. The experiment was conducted in a greenhouse during the period of August to December of 2015. It was done by utilizing four treatments: T1 = control substrate (50% soil and 50% commercial substrate Carolina Soil); T2 = vermicompost (40%) and control substrate (60%); T3 = Trichoderma sp. added to the control substrate; T4 = Trichoderma sp. added to the vermicompost (40%) and control substrate (60%). The gladiolus variety used was the Peter's Pears. The variables analyzed were main plant height, Falker chlorophyll index, length and diameter of the main stem, tassel length, quantity of main stem florets, proportionality between tassel and stem, and time until the start of flowering. It was concluded that the development and the quality of the gladiolus floral stem were favored by the use of vermicompost in the concentration of 40% added to the substrate.

https://doi.org/10.14295/oh.v24i1.1131 1260 downloads

15.

Diagnosis and Management of Classica Homocystinuria in Brazil: A Summary of 72 Late-Diagnosed Patients
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Poloni, Soraia

; Hoss, Giovana W.

; Sperb-Ludwig, Fernanda

; Borsatto, Taciane

; Doriqui, Maria Juliana R.

; Leão, Emília K.E.A

; Boa-Sorte, Ney

; Lourenço, Charles M.

; Kim, Chong A.

; Souza, Carolina F. M. de

; Rocha, Helio

; Ribeiro, Marcia

; Steiner, Carlos E.

; Moreno, Carolina A.

; Bernardi, Pricila

; Valadares, Eugenia

; Artigalas, Osvaldo

; Carvalho, Gerson

; Wanderley, Hector Y. C.

; D’Almeida, Vânia

; Santana-da-Silva, Luiz C.

; Blom, Henk J.

; Schwartz, Ida V. D.

Journal of Inborn Errors of Metabolism and Screening

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2018, Volume 6 elocation e180007

Abstract: En | Text: En | PDF: En

Abstract This study described a broad clinical characterization of classical homocystinuria (HCU) in Brazil. This was a cross-sectional, observational study including clinical and biochemical data from 72 patients (60 families) from Brazil (South, n = 13; Southeast, n = 37; Northeast, n = 8; North, n = 1; and Midwest, n = 1). Parental consanguinity was reported in 42% of families. Ocular manifestations were the earliest detected symptom (53% of cases), the main reason for diagnostic suspicion (63% of cases), and the most prevalent manifestation at diagnosis (67% of cases). Pyridoxine responsiveness was observed in 14% of patients. Only 22% of nonresponsive patients on treatment had total homocysteine levels <100 mmol/L. Most commonly used treatment strategies were pyridoxine (93% of patients), folic acid (90%), betaine (74%), vitamin B12 (27%), and low-methionine diet + metabolic formula (17%). Most patients diagnosed with HCU in Brazil are late diagnosed, express a severe phenotype, and poor metabolic control. Milder forms of HCU are likely underrepresented due to underdiagnosis.

https://doi.org/10.1177/2326409818788900 1352 downloads

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