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PR00893

Identifier
RABESCORT  [View Relations]  [View Alignment]  
Accession
PR00893
No. of Motifs
11
Creation Date
07-JUN-1998  (UPDATE 07-JUN-1999)
Title
Rab escort (choroideraemia) protein signature
Database References
PRINTS; PR00891 RABGDIREP
INTERPRO; IPR001738
Literature References
1. ALEXANDROV, K., HORIUCHI, H., STEELE-MORTIMER, O., SEABRA, M.C.
AND ZERIAL, M.
RAB escort protein-1 is a multifunctional protein that accompanies newly
prenylated Rab proteins to their target membranes. 
EMBO J. 13 5262-5273 (1994). 
 
2. ANDRES, D.A., SEABRA, M.C., BROWN, M.S., ARMSTRONG, S.A., SMELAND, T.E.,
CREMERS, F.P. AND GOLDSTEIN, J.L.
cDNA cloning of component A of Rab geranylgeranyl transferase and
demonstration of its role as a Rab escort protein. 
CELL 73 1091-1099 (1993). 
 
3. CREMERS, F.P., VAN DE POL, D.J., VAN KERKHOFF, L.P., WIERINGA, B.
AND ROPERS, H.H.
Cloning of a gene that is rearranged in patients with choroideraemia.
NATURE 347 674-677 (1990). 
 
4. VAN BOKHOVEN, H., VAN DEN HURK, J.A., BOGERD, L., PHILIPPE, C.,
GILGENKRANTZ, S., DE JONG, P., ROPERS, H.H. AND CREMERS, F.P.
Cloning and characterization of the human choroideremia gene. 
HUM.MOL.GENET. 3 1041-1046 (1994). 
 
5. MERRY, D.E., JANNE, P.A., LANDERS, J.E., LEWIS, R.A. AND NUSSBAUM, R.L.
Isolation of a candidate gene for choroideremia. 
PROC.NATL.ACAD.SCI.U.S.A. 89 2135-2139 (1992). 

Documentation
Rab proteins constitute a family of small GTPases that serve a regulatory
role in vesicular membrane traffic [1,2]; C-terminal geranylgeranylation is
crucial for their membrane association and function. This post-translational
modification is catalysed by Rab geranylgeranyl transferase (Rab-GGTase), a 
multi-subunit enzyme that contains a catalytic heterodimer and an accessory
component, termed Rab escort protein (REP)-1 [1,2]. REP-1 presents newly-
synthesised Rab proteins to the catalytic component, and forms a stable
complex with the prenylated proteins following the transfer reaction. 
 
cDNA cloning of component A of rat Rab geranylgeranyl transferase (REP)
confirms its resemblance to Rab3A guanine nucleotide dissociation inhibitor
(GDI) and its identity with the human choroideremia gene product [2]. A
genetic defect in REP underlies human choroideremia. Choroideraemia 
(or tapetochoroidal dystrophy) is a common form of X-linked blindness
characterised by progressive dystrophy of the choroid, retinal pigment
epithelium and retina [3-5]. 
 
RABESCORT is an 11-element fingerprint that provides a signature for Rab
escort (choroideraemia) proteins. The fingerprint was derived from an
initial alignment of 3 sequences: the motifs were drawn from conserved
sections spanning the full alignment length, focusing on those regions that
characterise the REP proteins but distinguish them from the closely related
Rab GDI family. A single iteration on OWL30.2 was required to reach
convergence, no further sequences being identified beyond the starting set. 
A single partial match was found, A41937, a human choroideremia-linked
protein that lacks the portion of sequence bearing motifs 1-5.
 
An update on SPTR37_9f identified a true set of 3 sequences.
Summary Information
3 codes involving 11 elements
0 codes involving 10 elements
0 codes involving 9 elements
0 codes involving 8 elements
0 codes involving 7 elements
0 codes involving 6 elements
0 codes involving 5 elements
0 codes involving 4 elements
0 codes involving 3 elements
0 codes involving 2 elements
Composite Feature Index
1133333333333
1000000000000
900000000000
800000000000
700000000000
600000000000
500000000000
400000000000
300000000000
200000000000
1234567891011
True Positives
RAE1_HUMAN    RAE1_RAT      RAE2_HUMAN    
Sequence Titles
RAE1_HUMAN  RAB PROTEINS GERANYLGERANYLTRANSFERASE COMPONENT A 1 (RAB ESCORT PROTEIN 1) (REP-1) (CHOROIDERAEMIA PROTEIN) (TCD PROTEIN) - HOMO SAPIENS (HUMAN). 
RAE1_RAT RAB PROTEINS GERANYLGERANYLTRANSFERASE COMPONENT A 1 (RAB ESCORT PROTEIN 1) (REP-1) - RATTUS NORVEGICUS (RAT).
RAE2_HUMAN RAB PROTEINS GERANYLGERANYLTRANSFERASE COMPONENT A 2 (RAB ESCORT PROTEIN 2) (REP-2) (CHOROIDERAEMIA-LIKE PROTEIN) - HOMO SAPIENS (HUMAN).
Scan History
OWL30_2    1  100  NSINGLE    
SPTR37_9f 2 5 NSINGLE
Initial Motifs
Motif 1  width=19
Element Seqn Id St Int Rpt
GNWASFSFSGLLSWLKEYQ RAE1_HUMAN 45 45 -
GNWASFSFSGLLSWLKEYQ RAE1_RAT 45 45 -
GNWASFSFSGLLSWLKEYQ RAE2_HUMAN 45 45 -

Motif 2 width=18
Element Seqn Id St Int Rpt
WQDQILENEEAIALSRKD RAE1_HUMAN 75 11 -
WQEQILENEEAIPLSSKD RAE1_RAT 75 11 -
WQDLIHETEEAITLRKKD RAE2_HUMAN 76 12 -

Motif 3 width=21
Element Seqn Id St Int Rpt
TIQHVEVFCYASQDLHEDVEE RAE1_HUMAN 94 1 -
TIQHVEVFCYASQDLHKDVEE RAE1_RAT 94 1 -
TIQHTEAFPYASQDMEDNVEE RAE2_HUMAN 95 1 -

Motif 4 width=24
Element Seqn Id St Int Rpt
EAGALQKNHALVTSANSTEAADSA RAE1_HUMAN 114 -1 -
EAGALQKNHASVTSAQSAEAAEAA RAE1_RAT 114 -1 -
EIGALQKNPSLGVSNTFTEVLDSA RAE2_HUMAN 115 -1 -

Motif 5 width=17
Element Seqn Id St Int Rpt
NRIAYAQIIKEGRRFNI RAE1_HUMAN 213 75 -
NRITYSQIIKEGRRFNI RAE1_RAT 210 72 -
NRITYSQIVKEGRRFNI RAE2_HUMAN 215 76 -

Motif 6 width=19
Element Seqn Id St Int Rpt
MLMKFLTFCMEYEKYPDEY RAE1_HUMAN 294 64 -
MLMKFLTFCVEYEEHPDEY RAE1_RAT 291 64 -
MLMKFLTFCLEYEQHPDEY RAE2_HUMAN 296 64 -

Motif 7 width=17
Element Seqn Id St Int Rpt
TIDGLKATKNFLHCLGR RAE1_HUMAN 351 38 -
TVDGLKATKKFLQCLGR RAE1_RAT 348 38 -
TIDGLNATKNFLQCLGR RAE2_HUMAN 353 38 -

Motif 8 width=28
Element Seqn Id St Int Rpt
KESRKCKAIIDQFGQRIISEHFLVEDSY RAE1_HUMAN 411 43 -
KESRKCKAVIDQFGQRIISKHFIIEDSY RAE1_RAT 408 43 -
KESGRCKAIIDHFGQRINAKYFIVEDSY RAE2_HUMAN 413 43 -

Motif 9 width=20
Element Seqn Id St Int Rpt
KPRILWALYFNMRDSSDISR RAE1_HUMAN 543 104 -
KPRLLWALYFNMRDSSDISR RAE1_RAT 540 104 -
KPRLLWALYFNMRDSSGISR RAE2_HUMAN 545 104 -

Motif 10 width=21
Element Seqn Id St Int Rpt
YNDLPSNVYVCSGPDCGLGND RAE1_HUMAN 565 2 -
YNDLPSNVYVCSGPDSGLGND RAE1_RAT 562 2 -
YNGLPSNVYVCSGPDCGLGNE RAE2_HUMAN 567 2 -

Motif 11 width=20
Element Seqn Id St Int Rpt
PNEDFCPPPPNPEDIILDGD RAE1_HUMAN 600 14 -
PNEDFCPAPPNPEDIVLDGD RAE1_RAT 597 14 -
PTEEFCPPPPNPEDIIFDGD RAE2_HUMAN 602 14 -
Final Motifs
Motif 1  width=19
Element Seqn Id St Int Rpt
GNWASFSFSGLLSWLKEYQ RAE1_HUMAN 45 45 -
GNWASFSFSGLLSWLKEYQ RAE1_RAT 45 45 -
GNWASFSFSGLLSWLKEYQ RAE2_HUMAN 45 45 -

Motif 2 width=18
Element Seqn Id St Int Rpt
WQDQILENEEAIALSRKD RAE1_HUMAN 75 11 -
WQEQILENEEAIPLSSKD RAE1_RAT 75 11 -
WQDLIHETEEAITLRKKD RAE2_HUMAN 76 12 -

Motif 3 width=21
Element Seqn Id St Int Rpt
TIQHVEVFCYASQDLHEDVEE RAE1_HUMAN 94 1 -
TIQHVEVFCYASQDLHKDVEE RAE1_RAT 94 1 -
TIQHTEAFPYASQDMEDNVEE RAE2_HUMAN 95 1 -

Motif 4 width=24
Element Seqn Id St Int Rpt
EAGALQKNHALVTSANSTEAADSA RAE1_HUMAN 114 -1 -
EAGALQKNHASVTSAQSAEAAEAA RAE1_RAT 114 -1 -
EIGALQKNPSLGVSNTFTEVLDSA RAE2_HUMAN 115 -1 -

Motif 5 width=17
Element Seqn Id St Int Rpt
NRIAYAQIIKEGRRFNI RAE1_HUMAN 213 75 -
NRITYSQIIKEGRRFNI RAE1_RAT 210 72 -
NRITYSQIVKEGRRFNI RAE2_HUMAN 215 76 -

Motif 6 width=19
Element Seqn Id St Int Rpt
MLMKFLTFCMEYEKYPDEY RAE1_HUMAN 294 64 -
MLMKFLTFCVEYEEHPDEY RAE1_RAT 291 64 -
MLMKFLTFCLEYEQHPDEY RAE2_HUMAN 296 64 -

Motif 7 width=17
Element Seqn Id St Int Rpt
TIDGLKATKNFLHCLGR RAE1_HUMAN 351 38 -
TVDGLKATKKFLQCLGR RAE1_RAT 348 38 -
TIDGLNATKNFLQCLGR RAE2_HUMAN 353 38 -

Motif 8 width=28
Element Seqn Id St Int Rpt
KESRKCKAIIDQFGQRIISEHFLVEDSY RAE1_HUMAN 411 43 -
KESRKCKAVIDQFGQRIISKHFIIEDSY RAE1_RAT 408 43 -
KESGRCKAIIDHFGQRINAKYFIVEDSY RAE2_HUMAN 413 43 -

Motif 9 width=20
Element Seqn Id St Int Rpt
KPRILWALYFNMRDSSDISR RAE1_HUMAN 543 104 -
KPRLLWALYFNMRDSSDISR RAE1_RAT 540 104 -
KPRLLWALYFNMRDSSGISR RAE2_HUMAN 545 104 -

Motif 10 width=21
Element Seqn Id St Int Rpt
YNDLPSNVYVCSGPDCGLGND RAE1_HUMAN 565 2 -
YNDLPSNVYVCSGPDSGLGND RAE1_RAT 562 2 -
YNGLPSNVYVCSGPDCGLGNE RAE2_HUMAN 567 2 -

Motif 11 width=20
Element Seqn Id St Int Rpt
PNEDFCPPPPNPEDIILDGD RAE1_HUMAN 600 14 -
PNEDFCPAPPNPEDIVLDGD RAE1_RAT 597 14 -
PTEEFCPPPPNPEDIIFDGD RAE2_HUMAN 602 14 -