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Aspartoacylase
Aspartoacylase is a hydrolase, hydrolytic enzyme (, also called ''aminoacylase II'', ''ASPA'' and other names) that in humans is encoded by the ''ASPA'' gene. ASPA catalysis, catalyzes the deacylation of N-Acetylaspartate, ''N''-acetyl-l-aspartate (''N-acetylaspartate)'' into Aspartic acid, aspartate and acetate. It is a zinc-dependent hydrolase that promotes the deprotonation of water to use as a nucleophile in a mechanism analogous to many other zinc-dependent hydrolases. It is most commonly found in the brain, where it controls the levels of ''N''-acetyl-l-aspartate. Mutations that result in loss of aspartoacylase activity are associated with Canavan disease, a rare dominance (genetics), autosomal recessive Neurodegeneration, neurodegenerative disease. Structure Aspartoacylase is a Dimer (chemistry), dimer of two identical monomers of 313 amino acids and uses a zinc cofactor in each. There are two distinct domains in each monomer: the N-terminal domain from residues 1-212 a ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
ASPA Mechanism
ASPA may refer to: *''Allocation de Solidarité aux Personnes Agées'' *American Service-Members' Protection Act *American Society for Public Administration *American Samoa Power Authority *Animals (Scientific Procedures) Act 1986 *Antarctic Specially Protected Area *Aotearoa Student Press Association * ASPA (car), a 1920s Czech car *ASPA (gene), Aspartoacylase, on human chromosome 17 *Summit of South American-Arab Countries A summit is a point on a surface that is higher in elevation than all points immediately adjacent to it. The topographic terms acme, apex, peak (mountain peak), and zenith are synonymous. The term (mountain top) is generally used only for a m ... See also * Aspa (other) {{Disambiguation ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
N-Acetylaspartate
''N''-Acetylaspartic acid, or ''N''-acetylaspartate (NAA), is a derivative of aspartic acid with a formula of C6H9NO5 and a molecular weight of 175.139. NAA is the second-most-concentrated molecule in the brain after the amino acid glutamate. It is detected in the adult brain in neurons, oligodendrocytes and myelin and is synthesized in the Mitochondrion, mitochondria from the amino acid aspartic acid and Acetyl-CoA, acetyl-coenzyme A. Function The various functions served by NAA are under investigation, but the primary proposed functions include: * Neuronal osmolyte that is involved in fluid balance in the brain * Source of acetate for lipid and myelin synthesis in oligodendrocytes, the Neuroglia, glial cells that myelinate neuronal axons * Precursor for the synthesis of the neuronal dipeptide N-Acetylaspartylglutamate * Contributor to energy production from the amino acid glutamate in neuronal mitochondria In the brain, NAA was thought to be present predominantly in neur ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
ASPA Bound To An Intermediate Analogue
ASPA may refer to: *''Allocation de Solidarité aux Personnes Agées'' *American Service-Members' Protection Act *American Society for Public Administration *American Samoa Power Authority *Animals (Scientific Procedures) Act 1986 *Antarctic Specially Protected Area *Aotearoa Student Press Association * ASPA (car), a 1920s Czech car *ASPA (gene), Aspartoacylase, on human chromosome 17 *Summit of South American-Arab Countries A summit is a point on a surface that is higher in elevation than all points immediately adjacent to it. The topographic terms acme, apex, peak (mountain peak), and zenith are synonymous. The term (mountain top) is generally used only for a m ... See also * Aspa (other) {{Disambiguation ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Canavan Disease
Canavan disease, or Canavan-Van Bogaert-Bertrand disease, is a rare and fatal autosomal recessive degenerative disease that causes progressive damage to nerve cells and loss of white matter in the brain. It is one of the most common degenerative cerebral diseases of infancy. It is caused by a deficiency of the enzyme aminoacylase 2, and is one of a group of genetic diseases referred to as leukodystrophies. It is characterized by degeneration of myelin in the phospholipid layer insulating the axon of a neuron and is associated with a gene located on human chromosome 17. Symptoms and signs Symptoms of the most common (and most serious) form of Canavan disease typically appear in early infancy usually between the first three to six months of age. Canavan disease then progresses rapidly from that stage, with typical cases involving intellectual disability, loss of previously acquired motor skills, feeding difficulties, abnormal muscle tone (i.e., initial floppiness - hypotonia - that ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
ASPA Domains
ASPA may refer to: *''Allocation de Solidarité aux Personnes Agées'' *American Service-Members' Protection Act *American Society for Public Administration *American Samoa Power Authority *Animals (Scientific Procedures) Act 1986 *Antarctic Specially Protected Area *Aotearoa Student Press Association * ASPA (car), a 1920s Czech car *ASPA (gene), Aspartoacylase, on human chromosome 17 *Summit of South American-Arab Countries A summit is a point on a surface that is higher in elevation than all points immediately adjacent to it. The topographic terms acme, apex, peak (mountain peak), and zenith are synonymous. The term (mountain top) is generally used only for a m ... See also * Aspa (other) {{Disambiguation ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hydrolase
Hydrolase is a class of enzyme that commonly perform as biochemical catalysts that use water to break a chemical bond, which typically results in dividing a larger molecule into smaller molecules. Some common examples of hydrolase enzymes are esterases including lipases, phosphatases, glycosidases, peptidases, and nucleosidases. Esterases cleave ester bonds in lipids and phosphatases cleave phosphate groups off molecules. An example of crucial esterase is acetylcholine esterase, which assists in transforming the neuron impulse into the acetate group after the hydrolase breaks the acetylcholine into choline and acetic acid. Acetic acid is an important metabolite in the body and a critical intermediate for other reactions such as glycolysis. Lipases hydrolyze glycerides. Glycosidases cleave sugar molecules off carbohydrates and peptidases hydrolyze peptide bonds. Nucleosidases hydrolyze the bonds of nucleotides. Hydrolase enzymes are important for the body because they have degra ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Substrate (biochemistry)
In chemistry, the term substrate is highly context-dependent. Broadly speaking, it can refer either to a chemical species being observed in a chemical reaction, or to a surface on which other chemical reactions or microscopy are performed. In the former sense, a reagent is added to the ''substrate'' to generate a product through a chemical reaction. The term is used in a similar sense in synthetic and organic chemistry, where the substrate is the chemical of interest that is being modified. In biochemistry, an enzyme substrate is the material upon which an enzyme acts. When referring to Le Chatelier's principle, the substrate is the reagent whose concentration is changed. ;Spontaneous reaction : :*Where S is substrate and P is product. ;Catalysed reaction : :*Where S is substrate, P is product and C is catalyst. In the latter sense, it may refer to a surface on which other chemical reactions are performed or play a supporting role in a variety of spectroscopic and microscop ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
White Matter
White matter refers to areas of the central nervous system (CNS) that are mainly made up of myelinated axons, also called tracts. Long thought to be passive tissue, white matter affects learning and brain functions, modulating the distribution of action potentials, acting as a relay and coordinating communication between different brain regions. White matter is named for its relatively light appearance resulting from the lipid content of myelin. However, the tissue of the freshly cut brain appears pinkish-white to the naked eye because myelin is composed largely of lipid tissue veined with capillaries. Its white color in prepared specimens is due to its usual preservation in formaldehyde. Structure White matter White matter is composed of bundles, which connect various grey matter areas (the locations of nerve cell bodies) of the brain to each other, and carry nerve impulses between neurons. Myelin acts as an insulator, which allows electrical signals to jump, rather than c ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Carbonyl
In organic chemistry, a carbonyl group is a functional group composed of a carbon atom double-bonded to an oxygen atom: C=O. It is common to several classes of organic compounds, as part of many larger functional groups. A compound containing a carbonyl group is often referred to as a carbonyl compound. The term carbonyl can also refer to carbon monoxide as a ligand in an inorganic or organometallic complex (a metal carbonyl, e.g. nickel carbonyl). The remainder of this article concerns itself with the organic chemistry definition of carbonyl, where carbon and oxygen share a double bond. Carbonyl compounds In organic chemistry, a carbonyl group characterizes the following types of compounds: Other organic carbonyls are urea and the carbamates, the derivatives of acyl chlorides chloroformates and phosgene, carbonate esters, thioesters, lactones, lactams, hydroxamates, and isocyanates. Examples of inorganic carbonyl compounds are carbon dioxide and carbonyl sulfide. ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Tetrahedral Carbonyl Addition Compound
A tetrahedral intermediate is a reaction intermediate in which the bond arrangement around an initially double-bonded carbon atom has been transformed from trigonal to tetrahedral. Tetrahedral intermediates result from nucleophilic addition to a carbonyl group. The stability of tetrahedral intermediate depends on the ability of the groups attached to the new tetrahedral carbon atom to leave with the negative charge. Tetrahedral intermediates are very significant in organic syntheses and biological systems as a key intermediate in esterification, transesterification, ester hydrolysis, formation and hydrolysis of amides and peptides, hydride reductions, and other chemical reactions. History One of the earliest accounts of the tetrahedral intermediate came from Rainer Ludwig Claisen in 1887. In the reaction of benzyl benzoate with sodium methoxide, and methyl benzoate with sodium benzyloxide, he observed a white precipitate which under acidic conditions yields benzyl benzoate, methyl ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Organic Acid Anhydride
An organic acid anhydride is an acid anhydride that is an organic compound. An acid anhydride is a compound that has two acyl groups bonded to the same oxygen atom. A common type of organic acid anhydride is a carboxylic anhydride, where the parent acid is a carboxylic acid, the formula of the anhydride being (RC(O))2O. Symmetrical acid anhydrides of this type are named by replacing the word ''acid'' in the name of the parent carboxylic acid by the word ''anhydride''. Thus, (CH3CO)2O is called ''acetic anhydride.'' Mixed (or unsymmetrical) acid anhydrides, such as acetic formic anhydride (see below), are known, whereby reaction occurs between two different carboxylic acids. Nomenclature of unsymmetrical acid anhydrides list the names of both of the reacted carboxylic acids before the word "anhydride" (for example, the dehydration reaction between benzoic acid and propanoic acid would yield "benzoic propanoic anhydride"). One or both acyl groups of an acid anhydride may also be ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Glutamic Acid
Glutamic acid (symbol Glu or E; the ionic form is known as glutamate) is an α-amino acid that is used by almost all living beings in the biosynthesis of proteins. It is a non-essential nutrient for humans, meaning that the human body can synthesize enough for its use. It is also the most abundant excitatory neurotransmitter in the vertebrate nervous system. It serves as the precursor for the synthesis of the inhibitory gamma-aminobutyric acid (GABA) in GABA-ergic neurons. Its molecular formula is . Glutamic acid exists in three optically isomeric forms; the dextrorotatory -form is usually obtained by hydrolysis of gluten or from the waste waters of beet-sugar manufacture or by fermentation.Webster's Third New International Dictionary of the English Language Unabridged, Third Edition, 1971. Its molecular structure could be idealized as HOOC−CH()−()2−COOH, with two carboxyl groups −COOH and one amino group −. However, in the solid state and mildly acidic water solutio ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
