{PDOC50967}
{PS50967; HRDC}
{BEGIN}
***********************
* HRDC domain profile *
***********************

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.

-Sequences known to belong to this class detected by the profile: ALL.
-Other sequence(s) detected in Swiss-Prot: NONE.
-Last update: March 2004 / First entry.

[ 1] Morozov V., Mushegian A.R., Koonin E.V., Bork P.
     "A putative nucleic acid-binding domain in Bloom's and Werner's
     syndrome helicases."
     Trends Biochem. Sci. 22:417-418(1997).
     PubMed=9397680
[ 2] von Kobbe C., Thomae N.H., Czyzewski B.K., Pavletich N.P., Bohr V.A.
     J. Biol. Chem. 278:52997-53006(2003).
[ 3] Liu Z., Macias M.J., Bottomley M.J., Stier G., Linge J.P., Nilges M.,
     Bork P., Sattler M.
     "The three-dimensional structure of the HRDC domain and implications
     for the Werner and Bloom syndrome proteins."
     Structure 7:1557-1566(1999).
     PubMed=10647186

--------------------------------------------------------------------------------
PROSITE is copyrighted by the SIB Swiss Institute of Bioinformatics and
distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives
(CC BY-NC-ND 4.0) License, see https://prosite.expasy.org/prosite_license.html
--------------------------------------------------------------------------------

{END}