Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is an inherited (genetic) condition that prevents the body from breaking down certain fats and turning them into energy. VLCAD is an enzyme that processes types of fat called very long-chain fatty acids.Preferred molecular test to diagnose or rule out very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency following clinical and/or biochemical presentation. Also refer to Acylcarnitine Quantitative Profile, Plasma (0040033), Carnitine Panel (0081110), and Organic Acids, Urine (0098389).The Vaisala Indigo510 Transmitter is perfect for tough industrial applications where precise measurements are required for one parameter at a time. Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is a condition that prevents the body from converting certain fats to energy, particularly during periods without food (fasting). Signs and symptoms of VLCAD deficiency typically appear during infancy or early childhood and can include low blood sugar (hypoglycemia), lack of energy (lethargy), and muscle weakness.Vaisala recently launched the new Indigo510 transmitter, which is compatible with all Indigo smart probes. The Indigo range is a flexible measurement platform that enables operators to pick and choose measurement parameters to suit their specific application.
Very Long Chain Acyl Coa Series Transmitters To
A simpler solution with single-probe support and without compromising the benefits of modularity was required by some customers.” says Juhani Lehto, Product Manager, Industrial Measurements at Vaisala.Attaching Indigo-compatible measurement probes to the Indigo510 transmitter is simple. “With the Indigo510 our customers can measure humidity, dew point, moisture in oil, temperature, carbon dioxide, and vaporised hydrogen peroxide with the assurance of reliability, accuracy, and the strong chain of data provided by the Indigo ecosystem. The new transmitter provides the quality and performance that Vaisala is renowned for in a more affordable, but equally robust package,” Lehto added.The Indigo510 transmitter extends the options for data visualisation, connectivity, supply voltage, and wiring in comparison to using a stand-alone probe moreover, operators also get regular platform and software updates.The unique and expanding Indigo range enables reliable and precise measurements in a wide range of industrial applications and environments.The Indigo range creates a modular ecosystem with interchangeable smart probes, rugged transmitters, and Vaisala’s intuitive Insight PC software. The probes can be deployed independently without a transmitter, or with the Indigo500 or Indigo200 series transmitters to expand their capabilities. Thanks to the modular plug-and-play design, the Indigo probes and transmitters are simple to install, opeate, and maintain. What does acyl-coa dehydrogenase, long-chain mean Information and.Objective: To explore the clinical features and variations of ACADVL gene in 9 neonates with very long chain acyl-coenzyme A dehydrogenase deficiency (VLCADD).
energy derivation by oxidation of organic compounds negative regulation of fatty acid oxidation negative regulation of fatty acid biosynthetic process very-long-chain-acyl-CoA dehydrogenase activity long-chain-acyl-CoA dehydrogenase activity
In mice, these two genes are in a head-to-head orientation, but they do not overlap. While DLG4 and VLCAD share common regulatory elements, they each have separate and distinct tissue-specific elements that confer their function. It has been shown that treatment with DEHP results in upregulation by the minimal promoter. VLCAD has interesting gene structure in humans, in that is located in a head-to-head structure with the DLG4 gene on Chromosome 17, and that the transcribed regions of these genes overlap. The ACADVL gene contains 20 exons, and is about 5.4 kb long. Alternative splicing results in multiple transcript variants encoding different isoforms.
The first is severe, with an early childhood onset and high mortality rate the most common symptom is this form is cardiomyopathy. Clinical significance ACADVL is linked with very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCADD), which has many symptoms, and typically presents as one of three phenotypes. In mice that have VLCAD deficiency, there is little to no protein hyperacetylation in the liver this implies that the VLCAD protein is also necessary for protein acetylation in this biological system. VLCAD is specific to very long-chain fatty acids, typically C16-acylCoA and longer.
The deficiency is treated systematically, but certain conditions such as fasting, myocardial irritation, dehydration, and high fat diets are avoided in attempt to prevent secondary complications. The disease is typically diagnosed by first performing tandem mass spectrometry on a blood sample of the patient during a period of stress, and then performing molecular genetic testing for the presence of the ACADVL gene. The final form presents in adulthood, and presents as isolated skeletal muscle involvement, rhabdomyolysis, and myoglobinuria, which is triggered by exercise or fasting.
National Center for Biotechnology Information, U.S. ^ "Human PubMed Reference:". ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000018574 - Ensembl, May 2017 ^ a b c GRCh38: Ensembl release 89: ENSG00000072778 - Ensembl, May 2017 ACADVL appears to interact with RPSA and GPHN.
National Library of Medicine. National Center for Biotechnology Information, U.S. ^ "Mouse PubMed Reference:".
"Overlapping gene structure of human VLCAD and DLG4". ^ a b Zhou C, Blumberg B (Feb 2003). Bibcode: 1995PNAS.9210496S. Proceedings of the National Academy of Sciences of the United States of America. "Molecular basis of human mitochondrial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhood". ^ Strauss AW, Powell CK, Hale DE, Anderson MM, Ahuja A, Brackett JC, Sims HF (Nov 1995).
The Journal of Clinical Investigation. "Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients". ^ Aoyama T, Souri M, Ushikubo S, Kamijo T, Yamaguchi S, Kelley RI, Rhead WJ, Uetake K, Tanaka K, Hashimoto T (Jun 1995). Doi: 10.1016/s0888-7543(03)00211-8. "Characterization of the bidirectional promoter region between the human genes encoding VLCAD and PSD-95". ^ Zhang LF, Ding JH, Yang BZ, He GC, Roe C (Dec 2003).
"Clear correlation of genotype with disease phenotype in very-long-chain acyl-CoA dehydrogenase deficiency". ^ Andresen BS, Olpin S, Poorthuis BJ, Scholte HR, Vianey-Saban C, Wanders R, Ijlst L, Morris A, Pourfarzam M, Bartlett K, Baumgartner ER, deKlerk JB, Schroeder LD, Corydon TJ, Lund H, Winter V, Bross P, Bolund L, Gregersen N (Feb 1999). "Mitochondrial protein acetylation is driven by acetyl-CoA from fatty acid oxidation".
IntAct Molecular Interaction Database. ^ "75 binary interactions found for search term ACADVL". Cite journal requires |journal= ( help) "Very Long-Chain Acyl-Coenzyme a Dehydrogenase Deficiency".
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