Excinuclease
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Excision endonuclease, also known as excinuclease or UV-specific endonuclease, is a
nuclease A nuclease (also archaically known as nucleodepolymerase or polynucleotidase) is an enzyme capable of cleaving the phosphodiester bonds between nucleotides of nucleic acids. Nucleases variously effect single and double stranded breaks in their ta ...
(enzyme) which excises a fragment of
nucleotides Nucleotides are organic molecules consisting of a nucleoside and a phosphate. They serve as monomeric units of the nucleic acid polymers – deoxyribonucleic acid (DNA) and ribonucleic acid (RNA), both of which are essential biomolecules w ...
during
DNA repair DNA repair is a collection of processes by which a cell identifies and corrects damage to the DNA molecules that encode its genome. In human cells, both normal metabolic activities and environmental factors such as radiation can cause DNA dam ...
. The excinuclease cuts out a fragment by hydrolyzing two
phosphodiester bond In chemistry, a phosphodiester bond occurs when exactly two of the hydroxyl groups () in phosphoric acid react with hydroxyl groups on other molecules to form two ester bonds. The "bond" involves this linkage . Discussion of phosphodiesters is ...
s, one on either side of the lesion in the DNA. This process is part of "
nucleotide excision repair Nucleotide excision repair is a DNA repair mechanism. DNA damage occurs constantly because of chemicals (e.g. intercalating agents), radiation and other mutagens. Three excision repair pathways exist to repair single stranded DNA damage: Nucle ...
", a mechanism that can fix specific types of damage to the DNA in the
G1 phase The G1 phase, gap 1 phase, or growth 1 phase, is the first of four phases of the cell cycle that takes place in eukaryotic cell division. In this part of interphase, the cell synthesizes mRNA and proteins in preparation for subsequent steps leadi ...
of the eukaryotic
cell cycle The cell cycle, or cell-division cycle, is the series of events that take place in a cell that cause it to divide into two daughter cells. These events include the duplication of its DNA (DNA replication) and some of its organelles, and subs ...
. Such damage may include thymine dimers created by UV rays as well as the bulky distortions in DNA caused by oxidized benzopyrenes from sources such as cigarette smoke. A deficiency of excinuclease occurs in a rare autosomal recessive disease called xeroderma pigmentosum. This disease can cause light-skin, extreme freckling and facial lesions, as well as preventing the repair of pyrimidine dimers. Diagnosis of this disease is done by measuring the enzyme's level in white blood cells in a blood sample. Symptoms in children include extreme ultraviolet, UV sensitivity, excessive freckle, freckling, multiple skin cancers and corneal ulcerations. Typically, these symptoms are seen during a child's first sun exposure.


Notes

DNA repair {{Enzyme-stub