Glycosaminoglycans
Mostrando 1-12 de 339 artigos, teses e dissertações.
-
1. DESENVOLVIMENTO NOS MÉTODOS DE GLICOSILAÇÃO: UMA CHAVE PARA ACESSAR SUAS APLICAÇÕES NA SÍNTESE DE MOLÉCULAS BIOATIVAS
Glycosylation reaction is an important class of reactions in organic chemistry, and the development of the method contributes to the synthesis of many biologically active compounds containing various glycoside bonds. Is arguably the most important, albeit challenging, reaction in the field of carbohydrate chemistry. Examples of the products of glycosylation
Quím. Nova. Publicado em: 2021-04
-
2. Successful therapeutic approach in a patient with elephantiasic pretibial myxedema,
Abstract Localized pretibial myxedema is a dermopathy whose treatment is a challenge in dermatology, occurring in 0.5-4% of patients with Graves’ disease. This autoimmune thyroid condition stimulates the production of hyaluronic acid and glycosaminoglycans that are deposited particularly in the pretibial region. Clinically, it presents as a localized, circ
An. Bras. Dermatol.. Publicado em: 2020-08
-
3. Left ventricular assessment in patients with mucopolysaccharidosis using conventional echocardiography and myocardial deformation by two-dimensional speckle-tracking method,
Resumo Objetivo: A mucopolissacaridose é uma doença genética rara, caracterizada por depósito intralisossômico de glicosaminoglicanos. O comprometimento cardiovascular é frequente. Sinais e sintomas cardíacos são subestimados pelo envolvimento da doença em outros órgãos. A terapia de reposição enzimática pode ser usada em mucopolissacaridose I
J. Pediatr. (Rio J.). Publicado em: 12/09/2019
-
4. Cicatrização: uso de matriz de colágeno
Resumo A matriz de colágeno Ologen TM é um novo agente antifibrótico composto por uma matriz porosa de atelocolageno tipo I e glicosaminoglicanos reticulados, que pode ser utilizado como uma alternativa para a modulação da cicatrização nas cirurgias. Por ser altamente poroso e biodegradável, este implante auxilia nos processos de reparação fisioló
Rev. bras.oftalmol.. Publicado em: 12/08/2019
-
5. Recommendations for Assessment and Management of Health-Related Quality of Life in Patients with Mucopolysaccharidoses in Latin America
Abstract Mucopolysaccharidoses (MPS) constitute a heterogeneous group of rare genetic disorders caused by enzymatic deficiencies that lead to the accumulation of glycosaminoglycans (GAGs). Clinical observations suggest a health-related impairment in quality of life in patients with MPS. Professionals with extensive experience in the care of patients with inb
J. inborn errors metab. screen.. Publicado em: 15/07/2019
-
6. Characterization of Decellularized Human Pericardium for Tissue Engineering and Regenerative Medicine Applications
Resumo Fundameto: O enxerto de pericárdio pode ser usado em muitos procedimentos de correção cirúrgica. Uma das estratégias da engenharia tecidual é o processo de descelularização. No entanto, embora esse processo diminua a resposta imunogênica, a descelularização pode modificar tanto o comportamento como a composição da matriz extracelular nat
Arq. Bras. Cardiol.. Publicado em: 27/06/2019
-
7. Mucopolysaccharidosis VI: Evaluation After 2 Years of Treatment
Abstract Introduction: Mucopolysaccharidosis VI (MPS VI) is the result of the absence of arylsulfatase B leading to the abnormal lysosomal accumulation of glycosaminoglycans. Two different phenotypes have been described to date, namely, rapidly progressive and slowly progressive. Aim: To present the evolution of a slowly progressive phenotype of MPS VI in
J. inborn errors metab. screen.. Publicado em: 19/06/2019
-
8. Obstructive Sleep Apnea in MPS: A Systematic Review of Pretreatment and Posttreatment Prevalence and Severity
Abstract The mucopolysaccharidoses (MPSs) are a group of inherited, metabolic disorders characterized by progressive multisystem accumulation of partially degraded glycosaminoglycans. This manifests with multilevel airway obstruction, presenting with obstructive sleep apnea (OSA). We systematically reviewed the literature to determine the severity and preval
J. inborn errors metab. screen.. Publicado em: 19/06/2019
-
9. Mucopolysaccharidosis: Caregiver Quality of Life
Abstract The mucopolysaccharidoses (MPSs) are a group of rare genetic diseases caused by a deficiency of specific enzymes involved in catabolism of glycosaminoglycans, which causes multisystem abnormalities. Quality of life (QoL) is directly associated with physical, mental, and psychological well-being and with social relationships, including family and fri
J. inborn errors metab. screen.. Publicado em: 19/06/2019
-
10. Expert Opinions on Managing Fertility and Pregnancy in Patients With Mucopolysaccharidosis
Abstract The mucopolysaccharidosis (MPS) disorders are rare genetic diseases caused by deficiencies in lysosomal enzymes involved in the degradation of glycosaminoglycans, leading to pulmonary, cardiac and neurological dysfunctions, skeletal anomalies, impaired vision, and/or hearing and shortened life spans. Whereas in the past, few individuals with MPS rea
J. inborn errors metab. screen.. Publicado em: 30/05/2019
-
11. Phenotype-oriented NGS panels for mucopolysaccharidoses: Validation and potential use in the diagnostic flowchart
Abstract Mucopolysaccharidosis (MPS) are a group of rare genetic disorders caused by deficiency in the activity of specific lysosomal enzymes required for the degradation of glycosaminoglycans (GAGs). A defect in the activity of these enzymes will result in the abnormal accumulation of GAGs inside the lysosomes of most cells, inducing progressive cellular da
Genet. Mol. Biol.. Publicado em: 11/04/2019
-
12. Outcomes of a Physician Survey on the Type, Progression, Assessment, and Treatment of Neurological Disease in Mucopolysaccharidoses
Abstract The mucopolysaccharidosis (MPS) disorders are a group of rare, inherited lysosomal storage disorders. In each of the 11 MPS (sub)types, deficiency in a specific lysosomal enzyme (1 of 11 identified enzymes) leads to accumulation of glycosaminoglycans, resulting in cell, tissue, and multi-organ dysfunction. There is great heterogeneity in the clinica
J. inborn errors metab. screen.. Publicado em: 28/02/2019