Mucopolysaccharidosis Study on Selenium Levels

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Questions and Answers

Match the following antioxidants with their types:

Glutathione (GSH) = Non-enzymatic antioxidant Superoxide dismutase (SOD) = Antioxidant enzyme Catalase (CAT) = Antioxidant enzyme Polyphenols = Non-enzymatic antioxidant

Match the following components with their roles in Glutathione Peroxidase (GPx) activity:

Selenium (Se) = Essential mineral for GPx Selenocysteine = Key amino acid in selenoproteins Reactive Oxygen Species (ROS) = Targets for GPx protection Glutathione (GSH) = Substrate for GPx reaction

Match the following methods with their application in measuring Selenium concentrations:

Hydride generation atomic absorption spectrometry (HG AAS) = Plasma concentration determination Freshly drawn blood samples = Biochemical assays Laboratory of Inborn Errors of Metabolism (LEIM) = Analysis of blood samples Recommended Dietary Allowance (RDA) = Selenium intake guidance

Match the following diseases associated with oxidative stress:

Cancer = Non-neurodegenerative lysosomal storage disease Cardiovascular disease = Common in oxidative stress Neurodegenerative diseases = Associated with cellular damage Selenium deficiency = Can exacerbate oxidative stress Signup and view all the answers

Match the following factors influencing GPx activity:

Dietary selenium = Direct influence on GPx functionality Oxidative stress = Increases demand for GPx Other antioxidants = Supportive role in cellular protection Age-related changes = May affect selenium absorption Signup and view all the answers

Match the following measurements related to GPx activity:

GSH/GSSG ratio = Indicator of oxidative stress Plasma concentrations of Se = Reflect selenium status GPx activity = Reflects antioxidant capacity Oxidative stress markers = Used to assess cellular damage Signup and view all the answers

Match the following benefits of selenium supplementation:

Enhances GPx activity = Protection against ROS Supports antioxidant defense = Fights oxidative stress May reduce cancer risk = Potential role in disease prevention Assists in hormone regulation = Influence on overall health Signup and view all the answers

Match the following antioxidants with their source:

Vitamins = Dietary sources like fruits and vegetables Bilirubin = Breakdown product of heme Melatonin = Hormonal source from sleep cycles Estrogens = Associated with reproductive health Signup and view all the answers

Match the following components with their associated methods of analysis:

Glutathione (GSH) = High performance liquid chromatography (HPLC) Selenium levels = Spectrophotometric analysis Glutathione peroxidase (GPx) activity = Reduced NADPH as marker Oxidized glutathione (GSSG) = Method from Galdieri et al. (2007) Signup and view all the answers

Match the following terms with their definitions:

GPx = An enzyme that utilizes glutathione to protect against oxidative stress GSH = A reduced form of glutathione GSSG = The oxidized form of glutathione ERT = Enzyme replacement therapy Signup and view all the answers

Match the following molecules with their roles in oxidative stress:

NADPH = Donates electrons to GPx Selenium = Acts as a cofactor for GPx Glutathione = Antioxidant that reduces harmful peroxides Oxidative stress = Condition resulting from an imbalance of free radicals and antioxidants Signup and view all the answers

Match the following methodologies with their characteristics:

HPLC = Analyzes plasma concentrations of GSH and GSSG Spectrophotometric analysis = Used to measure GPx activity Fluorescence detection = Method of detection in HPLC for glutathione Isocratic elution = A type of HPLC method used for analysis Signup and view all the answers

Match the following findings with the associated research focus:

Se supplementation = Investigation of its impact on MPS patients MPS I, II, VI = Conditions being studied in relation to selenium Plasma selenium levels = Evaluated as part of nutritional monitoring Glutathione ratio = Used to assess oxidative stress status Signup and view all the answers

Match the following elements with their associated conditions:

Reduced glutathione = Protects cells from oxidative damage Oxidized glutathione = Forms part of the oxidative stress evaluation Erythrocyte GPx = Enzyme activity measured to evaluate oxidative stress Selenoproteins = Proteins whose function is linked to selenium levels Signup and view all the answers

Match the following study components with their respective functions:

Reference Center for Inborn Errors of Metabolism = Where ERT was administered Fluorescence detection = Enhances sensitivity of HPLC Column C18 Luna = Type of column used for HPLC analysis 30 patients = Sample size for selenium level measurement Signup and view all the answers

Match the following researchers with their contributions to glutathione analysis:

Pfeiffer et al. (1999) = Developed the method for glutathione HPLC analysis Galdieri et al. (2007) = Described the method for measuring erythrocyte GSH HPLC researchers = Pioneered high performance liquid chromatography techniques Oxidative stress researchers = Focused on balance between antioxidants and free radicals Signup and view all the answers

Match the following measurements with their corresponding values at baseline for glutathione parameters:

Total Glutathione (GSHt) = $7.90 ext{ mmol/g Hb}$ Glutathione Peroxidase (GPx) Activity = $16.46 ext{ U/g Hb}$ GSH/GSSG Ratio = $2.3$ Oxidized Glutathione (GSSG) = Not provided Signup and view all the answers

Match the time points with their corresponding measurements for glutathione parameters after selenium supplementation:

Total Glutathione (GSHt) after 6 months = $5.76 ext{ mmol/g Hb}$ GSH/GSSG Ratio after 6 months = $0.58$ Glutathione Peroxidase (GPx) Activity after 6 months = $4.53 ext{ U/g Hb}$ Oxidized Glutathione (GSSG) = Not provided Signup and view all the answers

Match the following groups of patients with the respective types of Mucopolysaccharidosis (MPS):

MPS I = 13 patients MPS II = 9 patients MPS VI = 8 patients Total Patients = 30 patients Signup and view all the answers

Match the following statistical tests used in the study with their corresponding purposes:

Paired t-test = To compare baseline and post-supplementation values Retrospective study = To assess medical records of past patients Selenium level measurement = To evaluate deficiency before supplementation Glutathione parameter measurement = To determine oxidative stress levels Signup and view all the answers

Match the following outcomes with their results post-supplementation:

Total Glutathione (GSHt) after supplementation = Decreased from $7.90$ to $5.76 ext{ mmol/g Hb}$ GSH/GSSG Ratio after supplementation = Decreased from $2.3$ to $0.58$ Glutathione Peroxidase (GPx) Activity after supplementation = Decreased from $16.46$ to $4.53 ext{ U/g Hb}$ Selenium Levels = 28 patients (93.3%) were below normal range Signup and view all the answers

Match the following researchers with their affiliations:

José Araújo de Oliveira-Silva = CREIM, Universidade Federal de São Paulo Joyce Umbelino Pinto Yamamoto = LEIM, Universidade Federal de São Paulo Ana Maria Martins = CREIM, Universidade Federal de São Paulo Vânia D’Almeida = LEIM, Universidade Federal de São Paulo Signup and view all the answers

Match the following statements regarding selenium supplementation with their corresponding effects:

Over 90% of patients were deficient in selenium = 28 out of 30 patients Duration of supplementation = 12 months Assessment method for selenium = Plasma selenium level measurement Enzyme type focused in the study = Glutathione peroxidase activity Signup and view all the answers

Match the keywords from the study with their meanings:

Mucopolysaccharidosis = A group of inherited lysosomal storage disorders Oxidative Stress = An imbalance between free radicals and antioxidants Selenium = A trace element important for various biological processes Glutathione Peroxidase = An enzyme that reduces lipid hydroperoxides to their corresponding alcohols Signup and view all the answers

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Study Notes

Mucopolysaccharidosis (MPS) and Oxidative Stress

This study aims to investigate the levels of plasma selenium (Se), oxidative stress and the effect of Se supplementation in patients with MPS I, II and VI.
30 patients were recruited for the study: 13 with MPS I, 9 with MPS II, and 8 with MPS VI.
All patients were undergoing enzyme replacement therapy (ERT).
The study found that 28 out of 30 patients (93.3%) had Se levels below the normal range, suggesting Se deficiency.
Patients received Se supplementation for 12 months, with Se levels measured at 6 and 12 months.
The study also measured glutathione peroxidase (GPx) activity, total glutathione (GSHt), oxidized glutathione (GSSG), and reduced glutathione (GSH) at baseline and 6 months after Se supplementation.
The mean GSHt at baseline was 7.90 ± 2.36 mmol/g Hb. After Se supplementation, it was 5.76 ± 1.13 mmol/g Hb.
GSH/GSSG ratio at baseline was 2.3 ± 1.16, which decreased to 0.58 ± 0.38 after Se supplementation.
GPx activity was 16.46 ± 3.31 U/g Hb at baseline and decreased to 4.53 ± 4.92 U/g Hb after Se supplementation.
The changes in GPx activity and GSH/GSSG ratio were statistically significant, suggesting an impact of Se supplementation on oxidative stress markers.

Selenium Supplementation in MPS

Se supplementation was based on the recommended dietary allowance (RDA) for each patient's age, aiming to meet the mineral needs of 97-98% of the population.
Se was administered as a syrup daily.
Adherence to treatment was monitored weekly/monthly.

Blood Analytes and Measurement Techniques

Plasma Se concentrations were determined using hydride generation atomic absorption spectrometry (HG AAS).
Total glutathione (GSHt) and reduced glutathione (GSH) concentrations were analyzed using high-performance liquid chromatography (HPLC) with fluorescence detection.
Oxidized glutathione (GSSG) quantification was performed using a method previously described for measuring erythrocyte GSH, but without adding a reducing agent.
Erythrocyte GPx activity was spectrophotometrically analyzed using reduced nicotinamide adenine dinucleotide phosphate (NADPH) as a marker.

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