Gene/Proteome Database (LMPD)

LMPD ID
LMP012452
Gene ID
24942
Species
Rattus norvegicus (Rat)
Gene Name
choroideremia (Rab escort protein 1)
Gene Symbol
Chm
Alternate Names
rab proteins geranylgeranyltransferase component A 1; REP-1; choroidermia; rab escort protein 1; choroideremia protein homolog; choroideraemia protein homolog;
Chromosome
X
Map Location
Xq32
Summary
may function as Rab escort protein (REP); mutations in human homolog associated with choroideremia, a disease of retinal degeneration [RGD, Feb 2006]
Orthologs
View orthologs and multiple alignments for Chm

Proteins

rab proteins geranylgeranyltransferase component A 1
Refseq ID NP_058763
Protein GI 8393110
UniProt ID P37727
mRNA ID NM_017067
Length 650
MADNLPSDFDVIVIGTGLPESIIAAACSRSGQRVLHVDSRSYYGGNWASFSFSGLLSWLKEYQENNDVVTENSMWQEQILENEEAIPLSSKDKTIQHVEVFCYASQDLHKDVEEAGALQKNHASVTSAQSAEAAEAAETSCLPTAVEPLSMGSCEIPAEQSQCPGPESSPEVNDAEATGKKENSDAKSSTEEPSENVPKVQDNTETPKKNRITYSQIIKEGRRFNIDLVSQLLYSRGLLIDLLIKSNVSRYAEFKNITRILAFREGTVEQVPCSRADVFNSKQLTMVEKRMLMKFLTFCVEYEEHPDEYRAYEGTTFSEYLKTQKLTPNLQYFVLHSIAMTSETTSCTVDGLKATKKFLQCLGRYGNTPFLFPLYGQGELPQCFCRMCAVFGGIYCLRHSVQCLVVDKESRKCKAVIDQFGQRIISKHFIIEDSYLSENTCSRVQYRQISRAVLITDGSVLKTDADQQVSILAVPAEEPGSFGVRVIELCSSTMTCMKGTYLVHLTCMSSKTAREDLERVVQKLFTPYTEIEAENEQVEKPRLLWALYFNMRDSSDISRDCYNDLPSNVYVCSGPDSGLGNDNAVKQAETLFQQICPNEDFCPAPPNPEDIVLDGDSSQQEVPESSVTPETNSETPKESTVLGNPEEPSE

Gene Information

Entrez Gene ID
24942
Gene Name
choroideremia (Rab escort protein 1)
Gene Symbol
Chm
Species
Rattus norvegicus

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005968 IDA:UniProtKB C Rab-protein geranylgeranyltransferase complex
GO:0005829 ISS:UniProtKB C cytosol
GO:0005096 IEA:UniProtKB-KW F GTPase activator activity
GO:0017137 IDA:UniProtKB F Rab GTPase binding
GO:0004663 IEA:InterPro F Rab geranylgeranyltransferase activity
GO:0018344 IDA:UniProtKB P protein geranylgeranylation
GO:0006612 ISS:UniProtKB P protein targeting to membrane

Domain Information

InterPro Annotations

Accession Description
IPR018203 GDP_dissociation_inhibitor
IPR001738 Rab escort (choroideraemia) protein
IPR016664 Rab protein geranylgeranyltransferase component A, eukaryota

UniProt Annotations

Entry Information

Gene Name
choroideremia (Rab escort protein 1)
Protein Entry
RAE1_RAT
UniProt ID
P37727
Species
Rat

Comments

Comment Type Description
Function Substrate-binding subunit of the Rab geranylgeranyltransferase (GGTase) complex. Binds unprenylated Rab proteins and presents the substrate peptide to the catalytic component B composed of RABGGTA and RABGGTB, and remains bound to it after the geranylgeranyl transfer reaction. The component A is thought to be regenerated by transferring its prenylated Rab back to the donor membrane. Besides, a pre-formed complex consisting of CHM and the Rab GGTase dimer (RGGT or component B) can bind to and prenylate Rab proteins; this alternative pathway is proposed to be the predominant pathway for Rab protein geranylgeranylation. {ECO:0000269|PubMed:12356470, ECO:0000269|PubMed:12620235, ECO:0000269|PubMed:15186776, ECO:0000269|PubMed:1525821, ECO:0000269|PubMed:8513495}.
Interaction P09527:Rab7a; NbExp=2; IntAct=EBI-1039231, EBI-916225;
Similarity Belongs to the Rab GDI family
Subcellular Location Cytoplasm, cytosol {ECO:0000269|PubMed:12356470, ECO:0000269|PubMed:1525821, ECO:0000269|PubMed:8513495}.
Subunit Monomer. Heterotrimer composed of RABGGTA, RABGGTB and CHM; within this trimer, RABGGTA and RABGGTB form the catalytic component B, while CHM (component A) mediates Rab protein binding. Can associate with the Rab GGTase dimer (RGGT or component B) prior to Rab protein binding; the association is stabilized by geranylgeranyl pyrophosphate (GGpp). The CHM:RGGT:Rab complex is destabilized by GGpp. Interacts with RAB1A, RAB1B, RAB7A and RAB27A and mediates their prenylation. {ECO:0000269|PubMed:11389151, ECO:0000269|PubMed:11675392, ECO:0000269|PubMed:12356470, ECO:0000269|PubMed:12620235, ECO:0000269|PubMed:15186776, ECO:0000269|PubMed:1525821, ECO:0000269|PubMed:8513495}.
Tissue Specificity Most abundant in the heart, brain, and spleen. Lower levels seen in the lung, liver, muscle and kidney. Extremely low levels seen in the testis.

Identical and Related Proteins

Unique RefSeq proteins for LMP012452 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
8393110 RefSeq NP_058763 650 rab proteins geranylgeranyltransferase component A 1

Identical Sequences to LMP012452 proteins

Reference Database Accession Length Protein Name
GI:8393110 GenBank AAA87626.1 650 RAB geranylgeranyl transferase component A [Rattus norvegicus]
GI:8393110 SwissProt P37727.1 650 RecName: Full=Rab proteins geranylgeranyltransferase component A 1; AltName: Full=Choroideremia protein homolog; AltName: Full=Rab escort protein 1; Short=REP-1 [Rattus norvegicus]

Related Sequences to LMP012452 proteins

Reference Database Accession Length Protein Name
GI:8393110 GenBank AAA87626.1 650 RAB geranylgeranyl transferase component A [Rattus norvegicus]
GI:8393110 PDB 1LTX 650 Chain R, Structure Of Rab Escort Protein-1 In Complex With Rab Geranylgeranyl Transferase And Isoprenoid
GI:8393110 SwissProt P37727.1 650 RecName: Full=Rab proteins geranylgeranyltransferase component A 1; AltName: Full=Choroideremia protein homolog; AltName: Full=Rab escort protein 1; Short=REP-1 [Rattus norvegicus]