37 relations: Acetoacetic acid, Aerobic organism, Alkaptonuria, Amino acid, Aromaticity, Beta sheet, C-terminus, Carbocation, Catabolism, Chemical reaction, Enzyme, Eukaryote, Fumaric acid, Fumarylacetoacetate hydrolase, Hawkinsinuria, Herbicide, Homogentisic acid, Hydroxylation, Ion, Iron, N-terminus, NIH shift, Nitisinone, Orphan drug, Oxidative decarboxylation, Oxygen, Oxygenase, Phenylpyruvic acid, Plastoquinone, Redox, Tocopherol, Type I tyrosinemia, Tyrosine, Tyrosinemia type III, X-ray crystallography, 4-hydroxyphenylpyruvate dioxygenase inhibitor, 4-Hydroxyphenylpyruvic acid.
Acetoacetic acid (also diacetic acid) is the organic compound with the formula CH3COCH2COOH.
An aerobic organism or aerobe is an organism that can survive and grow in an oxygenated environment.
Alkaptonuria is a rare inherited genetic disorder in which the body cannot process the amino acids phenylalanine and tyrosine, which occur in protein.
Amino acids are organic compounds containing amine (-NH2) and carboxyl (-COOH) functional groups, along with a side chain (R group) specific to each amino acid.
In organic chemistry, the term aromaticity is used to describe a cyclic (ring-shaped), planar (flat) molecule with a ring of resonance bonds that exhibits more stability than other geometric or connective arrangements with the same set of atoms.
The β-sheet (also β-pleated sheet) is a common motif of regular secondary structure in proteins.
The C-terminus (also known as the carboxyl-terminus, carboxy-terminus, C-terminal tail, C-terminal end, or COOH-terminus) is the end of an amino acid chain (protein or polypeptide), terminated by a free carboxyl group (-COOH).
A carbocation (/karbɔkətaɪː'jɔ̃/) is an ion with a positively charged carbon atom.
Catabolism (from Greek κάτω kato, "downward" and βάλλειν ballein, "to throw") is the set of metabolic pathways that breaks down molecules into smaller units that are either oxidized to release energy or used in other anabolic reactions.
A chemical reaction is a process that leads to the transformation of one set of chemical substances to another.
Enzymes are macromolecular biological catalysts.
Eukaryotes are organisms whose cells have a nucleus enclosed within membranes, unlike Prokaryotes (Bacteria and other Archaea).
Fumaric acid or trans-butenedioic acid is the chemical compound with the formula HO2CCH.
Fumarylacetoacetase is an enzyme that in humans is encoded by the FAH gene located on chromosome 15.
Hawkinsinuria, also called 4-Alpha-hydroxyphenylpyruvate hydroxylase deficiency, is an autosomal dominant metabolic disorder affecting the metabolism of tyrosine.
Herbicides, also commonly known as weedkillers, are chemical substances used to control unwanted plants.
Homogentisic acid (2,5-dihydroxyphenylacetic acid) is a phenolic acid usually found in Arbutus unedo (strawberry-tree) honey.
Hydroxylation is a chemical process that introduces a hydroxyl group (-OH) into an organic compound.
An ion is an atom or molecule that has a non-zero net electrical charge (its total number of electrons is not equal to its total number of protons).
Iron is a chemical element with symbol Fe (from ferrum) and atomic number 26.
The N-terminus (also known as the amino-terminus, NH2-terminus, N-terminal end or amine-terminus) is the start of a protein or polypeptide referring to the free amine group (-NH2) located at the end of a polypeptide.
An NIH shift is a chemical rearrangement where a hydrogen atom on an aromatic ring undergoes an intramolecular migration primarily during a hydroxylation reaction.
Nitisinone (INN), also known as NTBC (an abbreviation of its full chemical name) is a medication used to slow the effects of hereditary tyrosinemia type 1.
An orphan drug is a pharmaceutical agent that has been developed specifically to treat a rare medical condition, the condition itself being referred to as an orphan disease.
Oxidative decarboxylation reactions are oxidation reactions in which a carboxylate group is removed, forming carbon dioxide.
Oxygen is a chemical element with symbol O and atomic number 8.
An oxygenase is any enzyme that oxidizes a substrate by transferring the oxygen from molecular oxygen O2 (as in air) to it.
Phenylpyruvic acid is the organic compound with the formula C6H5CH2C(O)CO2H.
Plastoquinone (PQ) is an isoprenoid quinone molecule involved in the electron transport chain in the light-dependent reactions of photosynthesis.
Redox (short for reduction–oxidation reaction) (pronunciation: or) is a chemical reaction in which the oxidation states of atoms are changed.
Tocopherols (TCP) are a class of organic chemical compounds (more precisely, various methylated phenols), many of which have vitamin E activity.
Type 1 tyrosinemia, also known as hepatorenal tyrosinemia or tyrosinosis, is the most severe form of tyrosinemia, a buildup of too much of the amino acid tyrosine in the blood and tissues due to an inability to metabolize it.
Tyrosine (symbol Tyr or Y) or 4-hydroxyphenylalanine is one of the 20 standard amino acids that are used by cells to synthesize proteins.
Tyrosinemia type III is a rare disorder caused by a deficiency of the enzyme 4-hydroxyphenylpyruvate dioxygenase, encoded by the gene HPD.
X-ray crystallography is a technique used for determining the atomic and molecular structure of a crystal, in which the crystalline atoms cause a beam of incident X-rays to diffract into many specific directions.
4-Hydroxyphenylpyruvate dioxygenase (HPPD) inhibitors (HPPD inhbitors) are a class of herbicides that prevent plants by blocking 4-Hydroxyphenylpyruvate dioxygenase, an enzyme in plants that breaks down the amino acid, tyrosine into components that are used by plants to create other molecules that plants need.
4-Hydroxyphenylpyruvic acid (4-HPPA) is an intermediate in the metabolism of the amino acid phenylalanine.
4-hydroxyphenylpyruvate dioxygenase, 4-hydroxyphenylpyruvate:oxygen oxidoreductase (hydroxylating, decarboxylating), Alpha-Ketoisocaproate dioxygenase, EC 220.127.116.11, HPD (gene), KIC dioxygenase, KIC-dioxygenase, Ketoisocaproate dioxygenase, P-Hydroxyphenylpyruvate dioxygenase, Α-ketoisocaproate dioxygenase.