cystine/vnetje

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Biomarker for Cystinosis Disease: BioCystinosis (BioCystinosis)

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Prijava / prijava

Cystinosis is a rare, multisystem genetic disorder characterized by the accumulation of an amino acid called cystine in different tissues and organs of the body including the kidneys, eyes, muscles, liver, pancreas and brain. Generally, Cystinosis is broken down into three different forms known as

Therapeutic Effect of Recombinant Human Growth Hormone (rhGH) on the Myopathy of Cystinosis

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Prijava / prijava

Cystinosis is an autosomal recessive storage disorder due to defective transport of the amino acid cystine out of cellular lysosomes. Cystine accumulation leads to cellular dysfunction in most of organs and tissues. Available treatment with the cystine-depleting agent, cysteamine, can prevent or

Amino Acids Expression Profiling of Patients With Sepsis

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Regulating Urine pH Levels to Alleviate Chronic Joint Pain

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STUDY SYNOPSIS / Literature Review Inflammation is a local immune response to injury, infection and foreign molecules. Notwithstanding the provoking cause, inflammation is thought to have evolved as an adaptive response for restoring tissue homeostasis. The local inflammatory response causes

A Preliminary Study to Evaluate Cysteamine Therapy in Human Subjects With Non-Alcoholic Steatohepatitis (NASH)

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Prijava / prijava

Non-alcoholic fatty liver disease (NAFLD) steatohepatitis represents a spectrum of disease occurring in the absence of alcohol abuse. It is characterized by the presence of steatosis and may represent a hepatic manifestation of the metabolic syndrome (including obesity, diabetes and

Role of Antioxidants Supplementation in Chronic Pancreatitis

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INTRODUCTION Chronic pancreatitis (CP) is a progressive inflammatory disease of the pancreas accompanied by abdominal pain and in late stages, by exocrine and endocrine insufficiency. The etiology of CP include alcohol abuse, hereditary, ductal obstruction, tropical pancreatitis, systemic diseases

Cysteine Supplementation in Critically Ill Neonates

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Prijava / prijava

Specific Aims: Critically ill neonates have demonstrated low concentrations of the antioxidant glutathione and a high incidence of disease associated with oxidative injury compared to healthy neonates. Cysteine is considered to be a conditionally essential amino acid for neonates and is the rate

Effect of Sulfur Amino Acid Depletion and Acetaminophen on Plasma Glutatione

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Prijava / prijava

The objectives of this research are to evaluate toxicological perturbations of metabolism due to complex chemical mixtures and diet-chemical interactions using high-resolution 1H-NMR. These experiments are designed to provide data on the extent of normal variation in metabolic profiles within

Kidney Transplantation in Patients With Cystinosis

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Prijava / prijava

Cystinosis is an autosomal recessive disorder due to impaired cystine transport across the lysosomal membrane, resulting in abnormal cystine accumulation throughout the body. Fanconi syndrome manifests in the first year of life, and end-stage renal failure occurs at ~10 years of age, requiring

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