{PDOC50803}
{PS50803; OAR}
{BEGIN}
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* OAR domain profile *
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This 14  amino  acid motif has been identified within the C-terminal region of
several Paired-like homeodomain (HD) (see <PDOC00027>) containing proteins [1,
2]. It  was  named  OAR  domain after the initials of otp, aristaless, and rax
[3].

Although it  has  been  proposed  that  this  domain  could  be  important for
transactivation and be involved in protein-protein interactions or DNA binding
[3, 4], its function is not yet known.

Some proteins known to contain an OAR domain are listed below:

 - Human  RIEG.  Defects  in this protein are the cause of Rieger syndrome, an
   autosomal dominant disorder that includes anomalies of the anterior chamber
   of the eye, dental hypoplasia and a protuberant umbilicus [1].
 - Mammalian OG12X. The function of these proteins is not yet known [2].
 - Vertebrate  Rax,  encoded  by  the retina and anterior neural fold homeobox
   gene. This protein plays a role in the proliferation and/or differentiation
   of retinal cell [3].
 - Drosophila  DRX.  It could be a homolog of vertebrate Rax. It appears to be
   important in brain development [5].
 - Human  SHOX, encoded by the short stature homeobox-containing gene. Defects
   or lack  of this protein are the cause of short stature associated with the
   Turner syndrome [4].
 - Mammalian  PITX3.  This  protein  appears  to  be  involved  in  normal eye
   anterior-chamber and lens development [6,7]. In human, defects in PITX3 are
   associated with   anterior   segment   mesenchymal  dysgenesis  (ASMD)  and
   autosomal-dominant congenital cataracts (ADCC) [7].
 - Vertebrate Pax3 and Pax7, transcription factors that play fundamental roles
   in the development of multi-cellular animals (Metazoa) [8].
 - Vertebrate visual system homeobox 2 (VSX2) [8].
 - Vertebrate dorsal root ganglia homeobox protein (DRG11) [8].

The OAR domain has also been called paired-type homeodomain tail (PHT), paired
tail or C-peptide domain [8].

The profile we developed covers the entire OAR domain.

-Sequences known to belong to this class detected by the profile: ALL.
-Other sequence(s) detected in Swiss-Prot: NONE.
-Last update: April 2015 / Profile revised.

[ 1] Semina E.V., Reiter R., Leysens N.J., Alward W.L.M., Small K.W.,
     Datson N.A., Siegel-Bartelt J., Bierke-Nelson D., Bitoun P.,
     Zabel B.U., Carey J.C., Murray J.C.
     "Cloning and characterization of a novel bicoid-related homeobox
     transcription factor gene, RIEG, involved in Rieger syndrome."
     Nat. Genet. 14:392-399(1996).
     PubMed=8944018; DOI=10.1038/ng1296-392
[ 2] Semina E.V., Reiter R.S., Murray J.C.
     "A new human homeobox gene OGI2X is a member of the most conserved
     homeobox gene family and is expressed during heart development in
     mouse."
     Hum. Mol. Genet. 7:415-422(1998).
     PubMed=9466998
[ 3] Furukawa T., Kozak C.A., Cepko C.L.
     "rax, a novel paired-type homeobox gene, shows expression in the
     anterior neural fold and developing retina."
     Proc. Natl. Acad. Sci. U.S.A. 94:3088-3093(1997).
     PubMed=9096350
[ 4] Rao E., Weiss B., Fukami M., Rump A., Niesler B., Mertz A., Muroya K.,
     Binder G., Kirsch S., Winkelmann M., Nordsiek G., Heinrich U.,
     Breuning M.H., Ranke M.B., Rosenthal A., Ogata T., Rappold G.A.
     "Pseudoautosomal deletions encompassing a novel homeobox gene cause
     growth failure in idiopathic short stature and Turner syndrome."
     Nat. Genet. 16:54-63(1997).
     PubMed=9140395; DOI=10.1038/ng0597-54
[ 5] Eggert T., Hauck B., Hildebrandt N., Gehring W.J., Walldorf U.
     "Isolation of a Drosophila homolog of the vertebrate homeobox gene Rx
     and its possible role in brain and eye development."
     Proc. Natl. Acad. Sci. U.S.A. 95:2343-2348(1998).
     PubMed=9482887
[ 6] Semina E.V., Reiter R.S., Murray J.C.
     "Isolation of a new homeobox gene belonging to the Pitx/Rieg family:
     expression during lens development and mapping to the aphakia region
     on mouse chromosome 19."
     Hum. Mol. Genet. 6:2109-2116(1997).
     PubMed=9328475
[ 7] Semina E.V., Ferrell R.E., Mintz-Hittner H.A., Bitoun P., Alward W.L.M.,
     Reiter R.S., Funkhauser C., Daack-Hirsch S., Murray J.C.
     "A novel homeobox gene PITX3 is mutated in families with
     autosomal-dominant cataracts and ASMD."
     Nat. Genet. 19:167-170(1998).
     PubMed=9620774; DOI=10.1038/527
[ 8] Vorobyov E., Horst J.
     "Getting the proto-Pax by the tail."
     J. Mol. Evol. 63:153-164(2006).
     PubMed=16830101; DOI=10.1007/s00239-005-0163-7

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