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PROSITE documentation PDOC50967HRDC domain profile
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PURL: https://purl.expasy.org/prosite/documentation/PDOC50967
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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PROSITE method (with tools and information) covered by this documentation:
| 1 | Authors | Morozov V. Mushegian A.R. Koonin E.V. Bork P. |
| Title | A putative nucleic acid-binding domain in Bloom's and Werner's syndrome helicases. | |
| Source | Trends Biochem. Sci. 22:417-418(1997). | |
| PubMed ID | 9397680 |
| 2 | Authors | von Kobbe C. Thomae N.H. Czyzewski B.K. Pavletich N.P. Bohr V.A. |
| Source | J. Biol. Chem. 278:52997-53006(2003). |
| 3 | Authors | Liu Z. Macias M.J. Bottomley M.J. Stier G. Linge J.P. Nilges M. Bork P. Sattler M. |
| Title | The three-dimensional structure of the HRDC domain and implications for the Werner and Bloom syndrome proteins. | |
| Source | Structure 7:1557-1566(1999). | |
| PubMed ID | 10647186 |
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