Helicase, POLQ-like

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External IDsGeneCards: [1]
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Helicase, POLQ-like, also known as hel308 and Holliday junction migration protein, encoded by the gene HEL308, is a DNA helicase found in humans, archea and many other organisms.[1] Its principal function is to allow DNA replication to continue past DNA forks.[2]

Gene

The gene encoding this enzyme, HEL308, is located on chromosome 4q21 in humans.[3] It is associated with the polymerase pathway.[4]

Nomenclature

When first reported, Hel308 was called "Holliday junction migration protein." It is also called HelQ in Caenorhabditis elegans and humans.[5]

Like many proteins, Hel308 was named after a previously discovered protein to which it had some connection. In this case, the "Hel" stands for "human helicase" and the "308" is a reference to the Drosophila melanogaster protein Mus308, to which it is homologous.[5]

Classification

Hel308 is part of DNA helicase superfamily II, a group of enzymes that wind and unwind DNA.[1] Hel308 is found throughout archea and in some eukaryotes, including humans.[1][5] It contains twenty exons.[2]

Structure and function

Hel308's principal role is to repair replication-blocking lesions, such as interstrand DNA cross-links that interfere with the forking of DNA during replication.[1][5]

Hel308 is a large protein, 1101 amino acids in length,[3] with five separate domains. The third and fourth domains form a large central pore that holds single-stranded DNA. Its fifth domain acts as a brake by securing the single-strand DNA protruding through this pore.[6]

Clinical significance

Mutations in HEL308 are associated with cancer of the pharynx and mouth.[4]

See also

References

  1. 1.0 1.1 1.2 1.3 Tafel AA, Wu L, McHugh PJ (May 2011). "Human HEL308 localizes to damaged replication forks and unwinds lagging strand structures". The Journal of Biological Chemistry. 286 (18): 15832–40. doi:10.1074/jbc.M111.228189. PMC 3091193. PMID 21398521.
  2. 2.0 2.1 "HELQ helicase, POLQ-like [ Homo sapiens (human) ]". NCBI. National Institutes of Health. February 21, 2016.
  3. 3.0 3.1 Marini F, Wood RD (Mar 2002). "A human DNA helicase homologous to the DNA cross-link sensitivity protein Mus308". The Journal of Biological Chemistry. 277 (10): 8716–23. doi:10.1074/jbc.M110271200. PMID 11751861.
  4. 4.0 4.1 Babron MC, Kazma R, Gaborieau V, McKay J, Brennan P, Sarasin A, Benhamou S (Jul 2014). "Genetic variants in DNA repair pathways and risk of upper aerodigestive tract cancers: combined analysis of data from two genome-wide association studies in European populations". Carcinogenesis. 35 (7): 1523–7. doi:10.1093/carcin/bgu075. PMID 24658182.
  5. 5.0 5.1 5.2 5.3 Woodman IL, Bolt EL (Jan 2011). "Winged helix domains with unknown function in Hel308 and related helicases". Biochemical Society Transactions. 39 (1): 140–4. doi:10.1042/BST0390140. PMID 21265761.
  6. Richards JD, Johnson KA, Liu H, McRobbie AM, McMahon S, Oke M, Carter L, Naismith JH, White MF (Feb 2008). "Structure of the DNA repair helicase hel308 reveals DNA binding and autoinhibitory domains". The Journal of Biological Chemistry. 283 (8): 5118–26. doi:10.1074/jbc.M707548200. PMC 3434800. PMID 18056710.