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Protein

Gamma-aminobutyric acid receptor subunit rho-2

Gene

GABRR2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at transcript leveli

Functioni

GABA, the major inhibitory neurotransmitter in the vertebrate brain, mediates neuronal inhibition by binding to the GABA/benzodiazepine receptor and opening an integral chloride channel. Rho-2 GABA receptor could play a role in retinal neurotransmission.

GO - Molecular functioni

  • chloride channel activity Source: UniProtKB-KW
  • extracellular ligand-gated ion channel activity Source: InterPro
  • GABA-A receptor activity Source: ProtInc

GO - Biological processi

  • gamma-aminobutyric acid signaling pathway Source: Ensembl
  • ion transmembrane transport Source: Reactome
  • signal transduction Source: ProtInc
  • synaptic transmission Source: Reactome
  • transmembrane transport Source: Reactome
  • transport Source: ProtInc
  • visual perception Source: Ensembl
Complete GO annotation...

Keywords - Molecular functioni

Chloride channel, Ion channel

Keywords - Biological processi

Ion transport, Transport

Keywords - Ligandi

Chloride

Enzyme and pathway databases

ReactomeiREACT_25168. GABA A (rho) receptor activation.
REACT_25387. Ligand-gated ion channel transport.
SignaLinkiP28476.

Names & Taxonomyi

Protein namesi
Recommended name:
Gamma-aminobutyric acid receptor subunit rho-2
Alternative name(s):
GABA(A) receptor subunit rho-2
GABA(C) receptor
Gene namesi
Name:GABRR2
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640 Componenti: Chromosome 6

Organism-specific databases

HGNCiHGNC:4091. GABRR2.

Subcellular locationi

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Topological domaini21 – 260240ExtracellularCuratedAdd
BLAST
Transmembranei261 – 28121HelicalSequence AnalysisAdd
BLAST
Transmembranei294 – 31421HelicalSequence AnalysisAdd
BLAST
Transmembranei326 – 34621HelicalSequence AnalysisAdd
BLAST
Topological domaini347 – 44397CytoplasmicCuratedAdd
BLAST
Transmembranei444 – 46421HelicalSequence AnalysisAdd
BLAST

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Cell junction, Cell membrane, Membrane, Postsynaptic cell membrane, Synapse

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA28506.

Chemistry

DrugBankiDB00546. Adinazolam.
DB01558. Bromazepam.
DB01594. Cinolazepam.
DB01559. Clotiazepam.
DB00829. Diazepam.
DB01215. Estazolam.
DB01567. Fludiazepam.
DB00690. Flurazepam.
DB00801. Halazepam.
DB00683. Midazolam.
DB01595. Nitrazepam.
DB00842. Oxazepam.
DB01588. Prazepam.
DB01589. Quazepam.
DB00231. Temazepam.
DB00897. Triazolam.

Polymorphism and mutation databases

DMDMi410516956.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Signal peptidei1 – 2020Sequence AnalysisAdd
BLAST
Chaini21 – 465445Gamma-aminobutyric acid receptor subunit rho-2PRO_0000000488Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Glycosylationi120 – 1201N-linked (GlcNAc...)Sequence Analysis
Disulfide bondi178 ↔ 192By similarity
Glycosylationi254 – 2541N-linked (GlcNAc...)Sequence Analysis

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

PaxDbiP28476.
PRIDEiP28476.

PTM databases

PhosphoSiteiP28476.

Expressioni

Gene expression databases

BgeeiP28476.
GenevisibleiP28476. HS.

Organism-specific databases

HPAiHPA016467.

Interactioni

Subunit structurei

Generally pentameric. There are five types of GABA(A) receptor chains: alpha, beta, gamma, delta, and rho. Interacts with SQSTM1 (By similarity).By similarity

Protein-protein interaction databases

STRINGi9606.ENSP00000386029.

Structurei

3D structure databases

ProteinModelPortaliP28476.
SMRiP28476. Positions 64-353.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Signal, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiNOG77438.
GeneTreeiENSGT00760000118821.
HOGENOMiHOG000231335.
HOVERGENiHBG051707.
InParanoidiP28476.
KOiK05190.
OMAiDEHDFSM.
OrthoDBiEOG712TVZ.
TreeFamiTF315453.

Family and domain databases

Gene3Di2.70.170.10. 1 hit.
InterProiIPR006028. GABAA/Glycine_rcpt.
IPR008059. GABAAa_rho2_rcpt.
IPR008057. GABAAa_rho_rcpt.
IPR006202. Neur_chan_lig-bd.
IPR006201. Neur_channel.
IPR006029. Neurotrans-gated_channel_TM.
IPR018000. Neurotransmitter_ion_chnl_CS.
[Graphical view]
PANTHERiPTHR18945. PTHR18945. 1 hit.
PfamiPF02931. Neur_chan_LBD. 1 hit.
PF02932. Neur_chan_memb. 1 hit.
[Graphical view]
PRINTSiPR00253. GABAARECEPTR.
PR01670. GABAARRHO.
PR01672. GABAARRHO2.
PR00252. NRIONCHANNEL.
SUPFAMiSSF63712. SSF63712. 1 hit.
SSF90112. SSF90112. 1 hit.
TIGRFAMsiTIGR00860. LIC. 1 hit.
PROSITEiPS00236. NEUROTR_ION_CHANNEL. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative initiation. AlignAdd to basket

Isoform 1 (identifier: P28476-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MPYFTRLILF LFCLMVLVES RKPKRKRWTG QVEMPKPSHL YKKNLDVTKI
60 70 80 90 100
RKGKPQQLLR VDEHDFSMRP AFGGPAIPVG VDVQVESLDS ISEVDMDFTM
110 120 130 140 150
TLYLRHYWKD ERLAFSSASN KSMTFDGRLV KKIWVPDVFF VHSKRSFTHD
160 170 180 190 200
TTTDNIMLRV FPDGHVLYSM RITVTAMCNM DFSHFPLDSQ TCSLELESYA
210 220 230 240 250
YTDEDLMLYW KNGDESLKTD EKISLSQFLI QKFHTTSRLA FYSSTGWYNR
260 270 280 290 300
LYINFTLRRH IFFFLLQTYF PATLMVMLSW VSFWIDRRAV PARVSLGITT
310 320 330 340 350
VLTMTTIITG VNASMPRVSY VKAVDIYLWV SFVFVFLSVL EYAAVNYLTT
360 370 380 390 400
VQERKERKLR EKFPCMCGML HSKTMMLDGS YSESEANSLA GYPRSHILTE
410 420 430 440 450
EERQDKIVVH LGLSGEANAA RKKGLLKGQT GFRIFQNTHA IDKYSRLIFP
460
ASYIFFNLIY WSVFS
Length:465
Mass (Da):54,151
Last modified:October 31, 2012 - v5
Checksum:i209700077636A395
GO
Isoform 2 (identifier: P28476-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-1: M → MVKPGGICSATGYWKAAFCLTDVHKM

Note: Isoform 2 could be translated from an upstream initiator ATG located in frame within the first coding exon. The probability of a signal peptide within this isoform is very low. No experimental confirmation available.
Show »
Length:490
Mass (Da):56,817
Checksum:i18F3AF79BD2D2B13
GO

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti209 – 2091Y → H in AAA52510 (PubMed:1315307).Curated

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei1 – 11M → MVKPGGICSATGYWKAAFCL TDVHKM in isoform 2. CuratedVSP_044373

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M86868 mRNA. Translation: AAA52510.1.
AL121833 Genomic DNA. Translation: CAC07339.2.
BC130352 mRNA. Translation: AAI30353.1.
BC130354 mRNA. Translation: AAI30355.1.
CCDSiCCDS5020.3. [P28476-1]
PIRiA38079.
RefSeqiNP_002034.3. NM_002043.3. [P28476-1]
UniGeneiHs.719891.

Genome annotation databases

EnsembliENST00000402938; ENSP00000386029; ENSG00000111886. [P28476-1]
GeneIDi2570.
KEGGihsa:2570.
UCSCiuc003pnb.3. human. [P28476-1]

Keywords - Coding sequence diversityi

Alternative initiation

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M86868 mRNA. Translation: AAA52510.1.
AL121833 Genomic DNA. Translation: CAC07339.2.
BC130352 mRNA. Translation: AAI30353.1.
BC130354 mRNA. Translation: AAI30355.1.
CCDSiCCDS5020.3. [P28476-1]
PIRiA38079.
RefSeqiNP_002034.3. NM_002043.3. [P28476-1]
UniGeneiHs.719891.

3D structure databases

ProteinModelPortaliP28476.
SMRiP28476. Positions 64-353.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi9606.ENSP00000386029.

Chemistry

BindingDBiP28476.
ChEMBLiCHEMBL2375.
DrugBankiDB00546. Adinazolam.
DB01558. Bromazepam.
DB01594. Cinolazepam.
DB01559. Clotiazepam.
DB00829. Diazepam.
DB01215. Estazolam.
DB01567. Fludiazepam.
DB00690. Flurazepam.
DB00801. Halazepam.
DB00683. Midazolam.
DB01595. Nitrazepam.
DB00842. Oxazepam.
DB01588. Prazepam.
DB01589. Quazepam.
DB00231. Temazepam.
DB00897. Triazolam.
GuidetoPHARMACOLOGYi421.

PTM databases

PhosphoSiteiP28476.

Polymorphism and mutation databases

DMDMi410516956.

Proteomic databases

PaxDbiP28476.
PRIDEiP28476.

Protocols and materials databases

DNASUi2570.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000402938; ENSP00000386029; ENSG00000111886. [P28476-1]
GeneIDi2570.
KEGGihsa:2570.
UCSCiuc003pnb.3. human. [P28476-1]

Organism-specific databases

CTDi2570.
GeneCardsiGC06M089967.
H-InvDBHIX0200918.
HGNCiHGNC:4091. GABRR2.
HPAiHPA016467.
MIMi137162. gene.
neXtProtiNX_P28476.
PharmGKBiPA28506.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiNOG77438.
GeneTreeiENSGT00760000118821.
HOGENOMiHOG000231335.
HOVERGENiHBG051707.
InParanoidiP28476.
KOiK05190.
OMAiDEHDFSM.
OrthoDBiEOG712TVZ.
TreeFamiTF315453.

Enzyme and pathway databases

ReactomeiREACT_25168. GABA A (rho) receptor activation.
REACT_25387. Ligand-gated ion channel transport.
SignaLinkiP28476.

Miscellaneous databases

ChiTaRSiGABRR2. human.
GeneWikiiGABRR2.
GenomeRNAii2570.
NextBioi10165.
PROiP28476.
SOURCEiSearch...

Gene expression databases

BgeeiP28476.
GenevisibleiP28476. HS.

Family and domain databases

Gene3Di2.70.170.10. 1 hit.
InterProiIPR006028. GABAA/Glycine_rcpt.
IPR008059. GABAAa_rho2_rcpt.
IPR008057. GABAAa_rho_rcpt.
IPR006202. Neur_chan_lig-bd.
IPR006201. Neur_channel.
IPR006029. Neurotrans-gated_channel_TM.
IPR018000. Neurotransmitter_ion_chnl_CS.
[Graphical view]
PANTHERiPTHR18945. PTHR18945. 1 hit.
PfamiPF02931. Neur_chan_LBD. 1 hit.
PF02932. Neur_chan_memb. 1 hit.
[Graphical view]
PRINTSiPR00253. GABAARECEPTR.
PR01670. GABAARRHO.
PR01672. GABAARRHO2.
PR00252. NRIONCHANNEL.
SUPFAMiSSF63712. SSF63712. 1 hit.
SSF90112. SSF90112. 1 hit.
TIGRFAMsiTIGR00860. LIC. 1 hit.
PROSITEiPS00236. NEUROTR_ION_CHANNEL. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "Identification of a putative gamma-aminobutyric acid (GABA) receptor subunit rho2 cDNA and colocalization of the genes encoding rho2 (GABRR2) and rho1 (GABRR1) to human chromosome 6q14-q21 and mouse chromosome 4."
    Cutting G.R., Curristin S., Zoghbi H., O'Hara B., Seldin M.F., Uhl G.R.
    Genomics 12:801-806(1992) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
    Tissue: Retina.
  2. "The DNA sequence and analysis of human chromosome 6."
    Mungall A.J., Palmer S.A., Sims S.K., Edwards C.A., Ashurst J.L., Wilming L., Jones M.C., Horton R., Hunt S.E., Scott C.E., Gilbert J.G.R., Clamp M.E., Bethel G., Milne S., Ainscough R., Almeida J.P., Ambrose K.D., Andrews T.D.
    , Ashwell R.I.S., Babbage A.K., Bagguley C.L., Bailey J., Banerjee R., Barker D.J., Barlow K.F., Bates K., Beare D.M., Beasley H., Beasley O., Bird C.P., Blakey S.E., Bray-Allen S., Brook J., Brown A.J., Brown J.Y., Burford D.C., Burrill W., Burton J., Carder C., Carter N.P., Chapman J.C., Clark S.Y., Clark G., Clee C.M., Clegg S., Cobley V., Collier R.E., Collins J.E., Colman L.K., Corby N.R., Coville G.J., Culley K.M., Dhami P., Davies J., Dunn M., Earthrowl M.E., Ellington A.E., Evans K.A., Faulkner L., Francis M.D., Frankish A., Frankland J., French L., Garner P., Garnett J., Ghori M.J., Gilby L.M., Gillson C.J., Glithero R.J., Grafham D.V., Grant M., Gribble S., Griffiths C., Griffiths M.N.D., Hall R., Halls K.S., Hammond S., Harley J.L., Hart E.A., Heath P.D., Heathcott R., Holmes S.J., Howden P.J., Howe K.L., Howell G.R., Huckle E., Humphray S.J., Humphries M.D., Hunt A.R., Johnson C.M., Joy A.A., Kay M., Keenan S.J., Kimberley A.M., King A., Laird G.K., Langford C., Lawlor S., Leongamornlert D.A., Leversha M., Lloyd C.R., Lloyd D.M., Loveland J.E., Lovell J., Martin S., Mashreghi-Mohammadi M., Maslen G.L., Matthews L., McCann O.T., McLaren S.J., McLay K., McMurray A., Moore M.J.F., Mullikin J.C., Niblett D., Nickerson T., Novik K.L., Oliver K., Overton-Larty E.K., Parker A., Patel R., Pearce A.V., Peck A.I., Phillimore B.J.C.T., Phillips S., Plumb R.W., Porter K.M., Ramsey Y., Ranby S.A., Rice C.M., Ross M.T., Searle S.M., Sehra H.K., Sheridan E., Skuce C.D., Smith S., Smith M., Spraggon L., Squares S.L., Steward C.A., Sycamore N., Tamlyn-Hall G., Tester J., Theaker A.J., Thomas D.W., Thorpe A., Tracey A., Tromans A., Tubby B., Wall M., Wallis J.M., West A.P., White S.S., Whitehead S.L., Whittaker H., Wild A., Willey D.J., Wilmer T.E., Wood J.M., Wray P.W., Wyatt J.C., Young L., Younger R.M., Bentley D.R., Coulson A., Durbin R.M., Hubbard T., Sulston J.E., Dunham I., Rogers J., Beck S.
    Nature 425:805-811(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  3. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).

Entry informationi

Entry nameiGBRR2_HUMAN
AccessioniPrimary (citable) accession number: P28476
Secondary accession number(s): A2BDE4, Q9H153
Entry historyi
Integrated into UniProtKB/Swiss-Prot: December 1, 1992
Last sequence update: October 31, 2012
Last modified: June 24, 2015
This is version 154 of the entry and version 5 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 6
    Human chromosome 6: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.