Advertisement

Topics

Study of Protein Translocation in Patients With Beta-Oxidation Disorders

2014-08-27 03:58:18 | BioPortfolio

Summary

OBJECTIVES:

I. Characterize inheritance patterns of mutations in patients with beta-oxidation disorders.

Description

PROTOCOL OUTLINE:

Patients undergo clinical and molecular analysis of beta-oxidation enzyme metabolism. The evaluation includes a urinary metabolite profile, and DNA and familial studies.

Study Design

Primary Purpose: Screening

Conditions

Beta-Oxidation Disorder

Status

Active, not recruiting

Source

Office of Rare Diseases (ORD)

Results (where available)

View Results

Links

Published on BioPortfolio: 2014-08-27T03:58:18-0400

Clinical Trials [1018 Associated Clinical Trials listed on BioPortfolio]

Hepatic Glycogen and Fat Oxidation

Excessive fat in the liver is associated with impairments in metabolic health. Low levels of DNL and high levels of hepatic fat oxidation are considered to be protective. A decrease in gl...

Fatty Acid Oxidation Defects and Insulin Sensitivity

The purpose of this study is to learn more about what causes insulin resistance. It has been suggested that proper breakdown of fat into energy (oxidation) in the body is important to all...

Study of Triheptanoin for Treatment of Long-Chain Fatty Acid Oxidation Disorder

Humans eat long-chain fat in their diet and use it for energy during exercise and during periods of fasting. Patients with long-chain fatty acid oxidation disorders cannot use dietary fat ...

Determinants and Within-subject Variability of Maximal Fat Oxidation During Exercise in Adults

The ability to upregulate fat oxidation at appropriate times such as during fasting, low to moderate intensity exercise and after a high fat meal, is an important marker of health and a de...

Investigating the Effects of Beta-Hydroxy-Beta-Methylbutyrate on Glucose Handling in Older and Younger Men.

We intend to examine the effect of a commercially available nutritional supplement, Beta-hydroxy-beta-methylbutyrate(HMB) on whole-body responses to a sugar load.

PubMed Articles [7706 Associated PubMed Articles listed on BioPortfolio]

Efficacy of polymers from spontaneous carotenoid oxidation in reducing necrotic enteritis in broilers.

This study evaluated the preventive effect of the spontaneous oxidation of β-carotene (OxC-beta) in broiler chickens with necrotic enteritis by Clostridium perfringens taking into consideration vario...

Effects of cognitive-behavioral therapy on neurotrophic factors in patients with major depressive disorder.

To correlate neurotrophic factors - brain-derived neurotrophic factor (BDNF), glial cell line-derived neurotrophic factor (GDNF), and beta-nerve growth factor (beta-NGF) - and severity of depressive s...

Better Renal Resistive Index Profile in Subjects with Beta Thalassemia Minor


.

Beta thalassaemia minor is a common genetic disorder without any characteristic symptoms except mild anemia. It is found to be associated with some cardiovascular risk factors such as insulin resistan...

Pro-inflammatory cytokines attenuate glucose-stimulated insulin secretion from INS-1E insulinoma cells by restricting mitochondrial pyruvate oxidation capacity - Novel mechanistic insight from real-time analysis of oxidative phosphorylation.

Pro-inflammatory cytokines cause pancreatic beta cell failure during the development of type 2 diabetes. This beta cell failure associates with mitochondrial dysfunction, but the precise effects of cy...

Beta-cypermethrin exposure affects female reproduction by enhancing oxidative stress in mice uterine tissue.

This study aimed to investigate the toxicity mechanism of beta-cypermethrin (beta-CYP) on fertility in female mice. Eighty female mice were randomly assigned to four groups of 20 mice each: one contro...

Medical and Biotech [MESH] Definitions

An amino acid formed in vivo by the degradation of dihydrouracil and carnosine. Since neuronal uptake and neuronal receptor sensitivity to beta-alanine have been demonstrated, the compound may be a false transmitter replacing GAMMA-AMINOBUTYRIC ACID. A rare genetic disorder, hyper-beta-alaninemia, has been reported.

Enzymes that catalyze the first step in the beta-oxidation of FATTY ACIDS.

A PEROXISOME-specific enzyme that catalyzes the hydration step of the beta-oxidation pathway.

An autosomal recessively inherited disorder characterized by the accumulation of intermediate-density lipoprotein (IDL or broad-beta-lipoprotein). IDL has a CHOLESTEROL to TRIGLYCERIDES ratio greater than that of VERY-LOW-DENSITY LIPOPROTEINS. This disorder is due to mutation of APOLIPOPROTEINS E, a receptor-binding component of VLDL and CHYLOMICRONS, resulting in their reduced clearance and high plasma levels of both cholesterol and triglycerides.

A group of enzymes that catalyze the reversible reduction-oxidation reaction of 20-hydroxysteroids, such as from a 20-ketosteroid to a 20-alpha-hydroxysteroid (EC 1.1.1.149) or to a 20-beta-hydroxysteroid (EC 1.1.1.53).

More From BioPortfolio on "Study of Protein Translocation in Patients With Beta-Oxidation Disorders"

Advertisement
Quick Search
Advertisement
Advertisement

 

Relevant Topics

Enzymes
Enzymes are proteins that catalyze (i.e., increase the rates of) chemical reactions. In enzymatic reactions, the molecules at the beginning of the process, called substrates, are converted into different molecules, called products. Almost all chemical re...

Urology
Benign Prostatic Hyperplasia (BPH) Erectile Dysfunction Urology Urology is the branch of medicine concerned with the urinary tract and diseases that affect it. Examples include urethritis, urethrostenosis and incontinence. Urology is a su...

Bioinformatics
Bioinformatics is the application of computer software and hardware to the management of biological data to create useful information. Computers are used to gather, store, analyze and integrate biological and genetic information which can then be applied...


Searches Linking to this Trial