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PROSITE documentation PDOC50967 [for PROSITE entry PS50967]

HRDC domain profile





Description

The helicase and RNaseD C-terminal (HRDC) domain is an 80-amino acid domain usually found at the C-terminus of RecQ helicases and RNase D homologs from various organisms, including human, yeast and bacteria [1]. The HRDC domain is involved in the binding of DNA to specific DNA structures (e.g. long-forked duplexes and Holliday junctions) that are formed during replication, recombination or transcription [2].

The 3D structure of the HRDC domain consists of five helices (see <PDB:1D8B>). Conserved residues in all five helices are important for the packing of the HRDC fold [3].

Some proteins known to contain a HRDC domain are listed below:

  • Animal Werner syndrome helicase (WRN). In human, defects in WRN are the cause of Werner syndrome (WS), a rare autosomal recessive progeroid syndrome characterized by the premature onset of multiple age-related disorders.
  • Animal Bloom's syndrome protein (EC 3.6.1.-) (BLM). In human, defects in BLM are the cause of Bloom syndrome (BS), an autosomal recessive disorder characterized by proportionate pre- and postnatal growth deficiency, sun- sensitive telangiectatic hypo- and hyperpigmented skin, predisposition to malignancy, and chromosomal instability.
  • Human PM-Scl autoantigen.
  • Yeast SGS1, the ortholog of BLM. It is required for chromosome segregation and interacts with topoisomerases I and II.

The profile we developed covers the entire HRDC domain.

Last update:

March 2004 / First entry.

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Technical section

PROSITE method (with tools and information) covered by this documentation:

HRDC, PS50967; HRDC domain profile  (MATRIX)


References

1AuthorsMorozov V. Mushegian A.R. Koonin E.V. Bork P.
TitleA putative nucleic acid-binding domain in Bloom's and Werner's syndrome helicases.
SourceTrends Biochem. Sci. 22:417-418(1997).
PubMed ID9397680

2Authorsvon Kobbe C. Thomae N.H. Czyzewski B.K. Pavletich N.P. Bohr V.A.
SourceJ. Biol. Chem. 278:52997-53006(2003).

3AuthorsLiu Z. Macias M.J. Bottomley M.J. Stier G. Linge J.P. Nilges M. Bork P. Sattler M.
TitleThe three-dimensional structure of the HRDC domain and implications for the Werner and Bloom syndrome proteins.
SourceStructure 7:1557-1566(1999).
PubMed ID10647186



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