Epub 2017 Oct 30. Review. JIMD Rep. 2015;22:11-6. doi: In the human body, aspartate is most frequently synthesized through the transamination of oxaloacetate. Mol Genet Metab. [17] The polyaspartate market comprises a small fraction (est. Current applications include biodegradable polymers (polyaspartic acid), low calorie sweeteners (aspartame), scale and corrosion inhibitors, and resins. Aspartate has many other biochemical roles. Genetics Home Reference has merged with MedlinePlus. [16] Around 75% of superabsorbent polymers are used in disposable diapers and an additional 20% is used for adult incontinence and feminine hygiene products. 2018 The L-isomer of Asp is one of the 22 proteinogenic amino acids, i.e., the building blocks of proteins. google_ad_client = "pub-6843438281922578"; When ammonia enters the circulatory system it acts as a highly toxic substance which can be harmful to the central nervous system and cause neural and brain disorders. Industrially, aspartate is produced by amination of fumarate catalyzed by L-aspartate ammonia-lyase. What is the prognosis of a genetic condition? This gene provides instructions for making an enzyme called asparagine synthetase. In 2014, the global market for aspartic acid was 39.3 thousand short tons (35.7 thousand tonnes)[14] or about $117 million annually[15] with potential areas of growth accounting for an addressable market[clarification needed] of $8.78 billion (Bn). Lu YF, Yao X, Hamdan FF, Pelak K, Reznik-Wolf H, Bar-Joseph I, Oz-Levi D, Lev D, Aspartic acid is not an essential amino acid, which means that it can be synthesized from central metabolic pathway intermediates in humans. google_ad_slot = "0258686924"; Groups at risk of aspartic acid deficiency. Mutations in the ASNS gene that cause asparagine synthetase deficiency lead to a decrease or loss of functional enzyme. In addition to SAP, aspartic acid has applications in the $19Bn fertilizer industry, where polyaspartate improves water retention and nitrogen uptake;[18] the $1.1Bn (2020) concrete floor coatings market, where polyaspartic is a low VOC, low energy alternative to traditional epoxy resins;[19] and lastly the >$5Bn scale and corrosion inhibitors market.[20]. In plants and microorganisms, aspartate is the precursor to several amino acids, including four that are essential for humans: methionine, threonine, isoleucine, and lysine. Genetti CA, Agrawal PB, McKenna R, Kilberg MS. [8] Of these two forms, only one, "L-aspartic acid", is directly incorporated into proteins. In addition, aspartic acid acts as a hydrogen acceptor in a chain of ATP synthase. See our, URL of this page: https://medlineplus.gov/genetics/condition/asparagine-synthetase-deficiency/. Liver health - aspartic acid benefits the liver by removing excess ammonia from the liver. google_ad_width = 150; [7] Their original method used lead hydroxide, but various other acids or bases are now more commonly used instead. It is encoded by the codons GAU and GAC. Learn more. The superabsorbent polymers market is anticipated to grow at a compound annual growth rate of 5.5% from 2014 to 2019 to reach a value of $8.78Bn globally. There are two forms or enantiomers of aspartic acid. Deficiency of asparagine synthetase causes congenital D-Aspartate is one of two D-amino acids commonly found in mammals.[3]. The conversion of aspartate to these other amino acids begins with reduction of aspartate to its "semialdehyde", O2CCH(NH2)CH2CHO. Aspartic acid has an average occurrence of about 5% in all proteins, so Asp-N is widely used in peptide mapping and proteomic analysis [4,14,15]. Identifying and Correcting an Aspartic Acid Deficiency. Polyaspartic acid, the polymerization product of aspartic acid, is a biodegradable substitute to polyacrylate. Aspartic acid was first discovered in 1827 by Auguste-Arthur Plisson and Étienne Ossian Henry[6] by hydrolysis of asparagine, which had been isolated from asparagus juice in 1806. Michaud JL, Goldstein DB. Aspartate also plays an important role in the urea cycle. [11] Asparagine is derived from aspartate via transamidation: (where GC(O)NH2 and GC(O)OH are glutamine and glutamic acid, respectively). Function, structure, and role in disease. How are genetic conditions treated or managed? Mar;123(3):317-325. doi: 10.1016/j.ymgme.2017.12.433. Aspartic acid and health . However, extreme fatigue or depression can be indicators of a lack of naturally generated aspartic acid. [16] The three largest market segments include the U.S., Western Europe, and China. [12], Click on genes, proteins and metabolites below to link to respective articles. Your body is capable of producing its own supply of aspartic acid. The exact effect of asparagine synthetase deficiency on brain cells is unknown, but because of the severe features of this condition, it is clear that asparagine is necessary for normal brain development. This substance is often used as an ingredient in testosterone-boosting supplements on the market. Most people with this condition have an unusually small head size (microcephaly) that worsens over time due to loss (atrophy) of brain tissue. This condition is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. L, Décarie JC, Blaser S, Banwell B, Joshi RB, He XP, Patry L, Silver RJ, Deficiency: New Inborn Errors of Metabolism. [5] Under physiological conditions (pH 7.4) in proteins the side chain usually occurs as the negatively charged aspartate form, −COO−. To use the sharing features on this page, please enable JavaScript. As it is converted back into aspartic acid, asparagine releases energy that brain and nervous system rells use for metabolism. Dietary L-aspartic acid has been shown to act as an inhibitor of Beta-glucuronidase, which serves to regulate enterohepatic circulation of bilirubin and bile acids. More than 20 affected individuals have been described in the medical literature. D-aspartic acid, sometimes also known as D-asparagine, can sometimes be labeled as D-aspartate. (C6H4(CO)2NC(CO2Et)2).[10]. However, aspartic acid is found in: InChI=1S/C4H7NO4/c5-2(4(8)9)1-3(6)7/h2H,1,5H2,(H,6,7)(H,8,9), InChI=1/C4H7NO4/c5-2(4(8)9)1-3(6)7/h2H,1,5H2,(H,6,7)(H,8,9), Deprotonated zwitterion (aspartate): O=C([O-])CC([NH3+])C(=O)[O-], Deprotonated zwitterion (aspartate): C(C(C(=O)[O-])[NH3+])C(=O)[O-], Except where otherwise noted, data are given for materials in their. Asparagine synthetase deficiency is thought to be a rare condition. Asparagine, created from another amino acid, aspartic acid, is needed to maintain balance in the central nervous system; it prevents you from being either overly nervous or overly calm. Zhao H(1), Roistacher DM(1), Helmann JD(1). Asparagine synthetase deficiency is a condition that causes neurological problems in affected individuals starting soon after birth. J, Miller D, Zhu Y, Macintosh R, Dinger ME, Cowley MJ, Buckley MF, Roscioli T, proliferation of cells under conditions of limited asparagine. Aspartic acid deficiency decreases cellular energy and may likely be a factor in chronic fatigue. In addition to being a component of proteins, asparagine helps to break down toxic ammonia within cells, is important for protein modification, and is needed for making a molecule that transmits signals in the brain (a neurotransmitter). 16;80(2):429-41. doi: 10.1016/j.neuron.2013.08.013. [5] It is a non-essential amino acid in humans, meaning the body can synthesize it as needed. Assiri H, Alqahtani AA, Alaamery M, Rolfs A, Eyaid W. Asparagine Synthetase As a result, brain cells in people with asparagine synthetase deficiency have a shortage (deficiency) of this amino acid. Most individuals with asparagine synthetase deficiency have exaggerated reflexes (hyperreflexia) and weak muscle tone (hypotonia). Symptoms of aspartic acid are very rare, but may include fatigue and depression. Asparagine Synthetase Deficiency causes reduced The most common types involve a loss of consciousness, muscle rigidity, and convulsions (tonic-clonic); involuntary muscle twitches (myoclonic); or abnormal muscle contraction (tonic). Not all affected people experience the same type of seizure. Aspartic acid has an acidic side chain (CH2COOH) which reacts with other amino acids, enzymes and proteins in the body. Epub 2017 Dec 20. The biosynthesis of aspartate is facilitated by an aminotransferase enzyme: the transfer of an amine group from another molecule such as alanine or glutamine yields aspartate and an alpha-keto acid.[5]. variant in siblings with Asparagine Synthetase Deficiency. Aspartate deficiency limits peptidoglycan synthesis and sensitizes cells to antibiotics targeting cell wall synthesis in Bacillus subtilis. Asp is pervasive in biosynthesis. Symptoms of aspartic acid are very rare, but may include fatigue and depression. Symptoms of aspartic acid deficiency . People with asparagine synthetase deficiency typically do not survive past childhood. Because aspartate can be synthesized by the body it is classified as a non-essential amino acid. Aspartic acid (symbol Asp or D;[4] the ionic form is known as aspartate), is an α-amino acid that is used in the biosynthesis of proteins. Palmer EE, Hayner J, Sachdev R, Cardamone M, Kandula T, Morris P, Dias KR, Tao Genetic Testing Registry: Asparagine synthetase deficiency, National Organization for Rare Disorders (NORD). 2017 Dec Where enzymatic synthesis will produce one or the other, most chemical syntheses will produce both forms, "DL-aspartic acid", known as a racemic mixture. Sacharow SJ, Dudenhausen EE, Lomelino CL, Rodan L, El Achkar CM, Olson HE, What are the different ways in which a genetic condition can be inherited? Lerman-Sagie T, Leshinsky-Silver E, Anikster Y, Ben-Asher E, Olender T, Colleaux Epub 2015 Feb 8. Many affected individuals also have recurrent seizures (epilepsy). Global concrete floor coatings market will be worth US$1.1Bn by 2020. U.S. Department of Health and Human Services, congenital microcephaly-severe encephalopathy-progressive cerebral atrophy syndrome. Asparagine synthetase deficiency is caused by mutations in a gene called ASNS. Some affected individuals have blindness due to impairment of the area of the brain responsible for processing vision, called the occipital cortex (cortical blindness). In the urea cycle, aspartate and ammonia donate amino groups leading to the formation of urea. Bye A, Kilberg MS, Kirk EP. (2014). Aspartic acid is found in abundance in plant proteins, especially in sprouting seeds. Corrosion inhibitors market analysis by product, by application, by end-use industry, and segment forecasts to 2020. [9], Racemic aspartic acid can be synthesized from diethyl sodium phthalimidomalonate, In proteins aspartate sidechains are often hydrogen bonded to form asx turns or asx motifs, which frequently occur at the N-termini of alpha helices. Aspartic Acid. Dobrzeniecka S, Islam MS, Hasnat A, Samuels ME, Aryal DK, Rodriguiz RM, Jiang YH, One area of aspartic acid market growth is biodegradable superabsorbent polymers (SAP). Because aspartate can be synthesized by the body it is classified as a non-essential amino acid. Aspartic acid, like glutamic acid, is classified as an acidic amino acid, with a pKa of 3.9, however in a peptide this is highly dependent on the local environment, and could be as high as 14.

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