A5031804076- Lysosomal Storage Disorders Research
- Trypanosoma species research and implications
- Carbohydrate Chemistry and Synthesis
- Research on Leishmaniasis Studies
- Wastewater Treatment and Nitrogen Removal
- Agriculture Sustainability and Environmental Impact
- Protein Hydrolysis and Bioactive Peptides
- Insect Utilization and Effects
- Water Quality Monitoring Technologies
- Virus-based gene therapy research
- Marine Bivalve and Aquaculture Studies
- Food Waste Reduction and Sustainability
- Meat and Animal Product Quality
- Multi-Criteria Decision Making
- Water Quality Monitoring and Analysis
- Epigenetics and DNA Methylation
- Neurogenetic and Muscular Disorders Research
- Alkaline Phosphatase Research Studies
- Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
- Plant Ecology and Taxonomy Studies
- Coal and Coke Industries Research
- Environmental Science and Water Management
- Biomedical Research and Pathophysiology
- Glycogen Storage Diseases and Myoclonus
- Bone and Dental Protein Studies
Clinica Universidad de Navarra
2022-2024
Euskadiko Parke Teknologikoa
2018-2024
Texas A&M International University
2024
AZTI
2023
Northwestern University
2023
Guanacaste Conservation Area
2022
California State University, Fresno
2022
Institución Universitaria Escolme
2019
Tecnalia
2018
Texas A&M University
2014
Mucopolysaccharidosis IVA (MPS IVA; Morquio A disease) is an autosomal-recessive disorder caused by a deficiency of lysosomal N-acetylgalactosamine-6-sulfate sulfatase (GALNS; E.C.3.1.6.4). GALNS required to degrade glycosaminoglycans, keratan sulfate (KS), and chondroitin-6-sulfate. Accumulation undegraded substrates in lysosomes the affected tissues leads systemic bone dysplasia. We summarize information on 148 unique mutations determined date gene, including 26 novel (19 missense, four...
Mucopolysaccharidosis IVA (MPS IVA) is an autosomal recessive disorder caused by a deficiency of N-acetylgalactosamine-6-sulfate sulfatase (GALNS), leading to accumulation keratan sulfate (KS) and chrondroitin-6-sulfate. The pharmacokinetics biodistributions were determined for two recombinant human GALNSs produced in CHO cell lines: native GALNS sulfatase-modifier-factor 1 (SUMF1) modified GALNS. Preclinical studies enzyme replacement therapy (ERT) using enzymes performed on MPS mice....
<h3>Objective</h3> The aim of this study was to compare the incidence intra-operative and post-operative complications in open minimally invasive radical hysterectomy for patients with early-stage cervical cancer. <h3>Methods</h3> Data were collected from SUCCOR database 1272 stage IB1 cancer (International Federation Gynecology Obstetrics (FIGO), 2009) who underwent Europe between January 2013 December 2014. We reviewed duration surgeries, estimated blood loss, length hospital stay,...
Fish by-catches, along with other fish side-streams, were previously used as raw material for the production of fishmeal and oil but appropriate handling allows their use in more valuable options. The aim this research was to valorize undersized hake (Merluccius merluccius) a model using by-catch from Bay Biscay produce protein hydrolysates bioactivities. Six enzymes, different proteolytic activities (endo- or exoproteases) specificities, tested hydrolysates. Products obtained an...
Hereditary hemochromatosis (HH) is an autosomal recessive disease characterized by iron accumulation in several organs, followed organ damage and failure. The C282Y mutation the HFE gene explains 80-90% of all diagnosed cases HH populations northwestern European ancestry. Targeted disruption mouse Hfe (or introduction murine analogous to human mutation) produces a model HH. Another gene, H63D, more prevalent than C282Y. However, physiological consequences H63D (as well as C282Y/H63D compound...
Mucopolysaccharidosis IVA (MPS IVA) is an autosomal recessive disease caused by N-acetylgalactosamine-6-sulfate sulfatase (GALNS) deficiency. In recent studies of enzyme replacement therapy for animal models with lysosomal storage diseases, cellular and humoral immune responses to the injected enzymes have been recognized as major impediments effective treatment. To study long-term effectiveness side effects therapies in absence responses, we developed MPS mouse model, which has many...
This work aims to demonstrate that it is possible reduce the pollution of a food wastewater by promoting recovery valuable compounds as secondary raw material for other processes enhancing company sustainability. For this purpose, technical feasibility biomolecules present in mussel cooking side streams had been tested, through concentration and stabilization technologies obtain ingredients savoury use. Among all proteins from processing currents are available on market or those an advanced...
Growing demand in the fisheries sector has resulted a high generation of side-streams that are mainly treated as waste despite their potential value terms protein, fatty acids, and minerals. The WaSeaBi project, funded by EU under Horizon 2020 BBI JU initiative, seeks to address this problem promoting sustainable economically viable utilisation these side-streams, thus contributing improved food security environmental conservation. project focuses on development innovative technologies...
Brewer’s spent yeast (BSY) is produced by the beer industry and has high nutritional value great potential for producing high-value molecules, such as peptides, nutraceutical, food feed applications. In present research, Flavourzyme® Protamex® enzymes were selected protein hydrolysis based on previous studies. The optimum conditions enzymatic defined response surface methodology (RSM) Box–Behnken design composed of four variables: temperature, pH, enzyme dosage time. Protein content, degree...
Grape stem is a winery by-product that it currently disposed as waste or at best soil conditioner. However, rich in fibres and polyphenols which makes interesting for animal feeding. In this regard, rabbit farming emerges target livestock since fibre content essential rabbit’s diets preventing digestive troubles are associated with improved performances animals due to their antimicrobial antioxidant activities. This study aims assess the suitability of grape stem-based ingredient The was...
Mucopolysaccharidosis IVA (MPS IVA) is a lysosomal storage disorder caused by the deficiency of N-acetylgalactosamine-6-sulfate sulfatase (GALNS). Mutation screening GALNS gene was performed RT-PCR with one amplicon and direct sequence analyses using cDNA samples from 15 Italian MPS patients. Each mutation confirmed at genomic level. In this study, 13 different mutations four common (over 10% mutant alleles) were identified in 12 severe three milder (attenuated) The alterations out found to...
Testicular cell proliferation and differentiation is critical for development of normal testicular function male reproductive maturity. The objective the current study was to evaluate histoarchitecture expression genes marking specific cells important functions as well testosterone production developing goat testes. Testes were harvested from Alpine bucks at 0, 2, 4, 6, 8 mo age (n = 5/age group). Paired testes weight increased 2 4 (P < 0.001) 6 0.01). greatest increases in seminiferous...
Coral reefs in Culebra Bay (North Pacific of Costa Rica) are threatened by multiple anthropogenic disturbances including global warming, overfishing, eutrophication, and invasive species outbreaks. It is possible to assist their recovery implementing ecological restoration techniques. This study used artificial hexagonal steel structures, called “spiders” compare growth Pocillopora spp. coral fragments different sizes. Three initial fragment class sizes were used: 2, 5 8 cm, with each size...