Serum protein profile analysis in lysosomal storage disorders patients

Frutos, Laura López de; García-González, Elena; García-Rodríguez, Beatriz; González‐Irazabal, Yolanda; Lahoz, Carlos H.; Irún, Pilar; Cebolla, Jorge J.; Giraldo, Pilar

doi:10.1016/j.cca.2020.07.056

Cited by 3 publications

(1 citation statement)

References 41 publications

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“…Gaucher's disease, Hunter's syndrome, Fabry's disease, Hurler's syndrome, Morquio syndrome type A, Maroteaux-Lamy syndrome, Sly syndrome, α-mannosidosis, Batten disease and Pompe's disease are examples of disorders included in the LSD group. At the moment, some biomarker discovery projects are underway to improve LSD diagnosis [23,24]. Due to the features of the above-mentioned pathologies, ERT appears to be a promising therapeutic alternative.…”

Section: Lysosomal Storage Diseases (Lsd)mentioning

confidence: 99%

Enzyme Therapy: Current Challenges and Future Perspectives

Fuente

Lombardero²,

Gómez-González

et al. 2021

IJMS

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In recent years, enzymes have risen as promising therapeutic tools for different pathologies, from metabolic deficiencies, such as fibrosis conditions, ocular pathologies or joint problems, to cancer or cardiovascular diseases. Treatments based on the catalytic activity of enzymes are able to convert a wide range of target molecules to restore the correct physiological metabolism. These treatments present several advantages compared to established therapeutic approaches thanks to their affinity and specificity properties. However, enzymes present some challenges, such as short in vivo half-life, lack of targeted action and, in particular, patient immune system reaction against the enzyme. For this reason, it is important to monitor serum immune response during treatment. This can be achieved by conventional techniques (ELISA) but also by new promising tools such as microarrays. These assays have gained popularity due to their high-throughput analysis capacity, their simplicity, and their potential to monitor the immune response of patients during enzyme therapies. In this growing field, research is still ongoing to solve current health problems such as COVID-19. Currently, promising therapeutic alternatives using the angiotensin-converting enzyme 2 (ACE2) are being studied to treat COVID-19.

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Section: Lysosomal Storage Diseases (Lsd)mentioning

confidence: 99%

Enzyme Therapy: Current Challenges and Future Perspectives

Fuente

Lombardero²,

Gómez-González

et al. 2021

IJMS

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Recommendations for overcoming challenges in the diagnosis of lysosomal acid lipase deficiency

Giraldo

Frutos

Cebolla

2022

Expert Opinion on Orphan Drugs

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Effects of Dietary Thymoquinone Inclusion on Antioxidative, Oxidative, Pro-Inflammatory Responses, Semen Attributes and Testicular Changes in Heat-Stressed Rabbit Bucks

AlGaradi,

Sindi,

Al-Gabrif

et al. 2024

Annals of Animal Science

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Heat stress (HS) is considered a severe concern for rabbit buck’s health, welfare and reproductive features. Thymoquinone (THQ) is a natural molecule with robust antioxidant, antimicrobial, and anti-inflammatory properties. Thereafter, three months of feeding research was applied to assess the role of THQ-enriched diets in improving the health, semen attributes and testicular changes of stressed rabbit bucks. Forty rabbit bucks were used in this research, and animals were fed diets containing 0 (THQ0; serve as a heat stress group), 100 (THQ100), 200 (THQ200), and 400 (THQ400) mg/kg of THQ for three months continually during the summer season. Outcomes displayed that THQ significantly enhanced FBW (final body weight), DBWG (daily body weight gain), and FI (feed intake) but did not affect FCR (feed conversion ratio). Compared with the THQ0 group, dietary THQ addition significantly decreased creatinine, TB (total bilirubin), TG (triglycerides), and GGT (gamma-glutamyl transferase) levels. Bucks receiving 200 mg of THQ had higher TAC (total antioxidant capacity) and GSH (glutathione) than other groups. At the same time, MDA was significantly shrunk in THQ-treated groups relative to the THQ group. The levels of interferon-gamma (IFN-ᵞ) and interleukin-4 (IL-4) were significantly diminished (P <0.001), but nitric oxide and lysosome activity were significantly increased in bucks given diets containing THQ (200 and 400 mg/kg) as relative to the TH0 group. Libido score and reaction time were significantly improved by THQ dietary inclusion. Semen attributes, including volume, motility, concentration, and vitality, were significantly augmented in THQ100 and THQ200 groups. Histological screening of testicular tissues showed moderate to severe degenerative and necrotic changes in the testicular tissues of bucks exposed to HS. This was enhanced with the supplementation of THQ at different levels. Collectively, THQ (200 or 400mg /kg diet) can improve health, semen attributes and restore testicular damage in rabbit bucks via boosting antioxidant and reducing inflammatory cytokines.

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Serum protein profile analysis in lysosomal storage disorders patients

Cited by 3 publications

References 41 publications

Enzyme Therapy: Current Challenges and Future Perspectives

Enzyme Therapy: Current Challenges and Future Perspectives

Recommendations for overcoming challenges in the diagnosis of lysosomal acid lipase deficiency

Effects of Dietary Thymoquinone Inclusion on Antioxidative, Oxidative, Pro-Inflammatory Responses, Semen Attributes and Testicular Changes in Heat-Stressed Rabbit Bucks

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