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Protein

Glycine receptor subunit alpha-1

Gene

Glra1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Glycine receptors are ligand-gated chloride channels. Channel opening is triggered by extracellular glycine (PubMed:16672662, PubMed:17114051, PubMed:24801766). Channel opening is also triggered by taurine and beta-alanine (By similarity). Channel characteristics depend on the subunit composition; heteropentameric channels are activated by lower glycine levels and display faster desensitization (By similarity). Plays an important role in the down-regulation of neuronal excitability (PubMed:9145798). Contributes to the generation of inhibitory postsynaptic currents (PubMed:16672662, PubMed:17114051, PubMed:24801766). Channel activity is potentiated by ethanol. Potentiation of channel activity by intoxicating levels of ethanol contribute to the sedative effects of ethanol (PubMed:24801766).By similarity4 Publications

Miscellaneous

The alpha subunit binds strychnine.3 Publications

Enzyme regulationi

Inhibited by strychnine. Inhibited by picrotoxin (PubMed:16672662). Channel activity is enhanced by 5 µM Zn2+ and inhibited by 100 µM Zn2+ (PubMed:17114051).2 Publications

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi220ZincBy similarity1
Metal bindingi222ZincBy similarity1
Metal bindingi243ZincBy similarity1
Sitei289Important for obstruction of the ion pore in the closed conformationBy similarity1

GO - Molecular functioni

GO - Biological processi

  • acrosome reaction Source: MGI
  • action potential Source: MGI
  • adult walking behavior Source: MGI
  • anion transport Source: MGI
  • cellular response to amino acid stimulus Source: UniProtKB
  • cellular response to ethanol Source: UniProtKB
  • cellular response to zinc ion Source: UniProtKB
  • chemical synaptic transmission Source: MGI
  • chloride transmembrane transport Source: MGI
  • chloride transport Source: UniProtKB
  • inhibitory postsynaptic potential Source: UniProtKB
  • ion transport Source: UniProtKB
  • muscle contraction Source: UniProtKB
  • negative regulation of transmission of nerve impulse Source: UniProtKB
  • neuromuscular process Source: MGI
  • neuromuscular process controlling posture Source: MGI
  • neuropeptide signaling pathway Source: UniProtKB
  • positive regulation of acrosome reaction Source: MGI
  • protein heterooligomerization Source: MGI
  • protein homooligomerization Source: MGI
  • regulation of membrane potential Source: MGI
  • regulation of respiratory gaseous exchange Source: MGI
  • regulation of respiratory gaseous exchange by neurological system process Source: MGI
  • response to alcohol Source: UniProtKB
  • response to amino acid Source: GO_Central
  • righting reflex Source: MGI
  • startle response Source: MGI
  • synaptic transmission, glycinergic Source: UniProtKB
  • visual perception Source: MGI

Keywordsi

Molecular functionChloride channel, Ion channel, Ligand-gated ion channel, Receptor
Biological processIon transport, Transport
LigandChloride, Metal-binding, Zinc

Enzyme and pathway databases

ReactomeiR-MMU-112314 Neurotransmitter receptors and postsynaptic signal transmission

Names & Taxonomyi

Protein namesi
Recommended name:
Glycine receptor subunit alpha-1
Alternative name(s):
Glycine receptor 48 kDa subunit
Glycine receptor strychnine-binding subunit
Gene namesi
Name:Glra1
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 11

Organism-specific databases

MGIiMGI:95747 Glra1

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini29 – 250ExtracellularBy similarityAdd BLAST222
Transmembranei251 – 272Helical; Name=1By similarityAdd BLAST22
Topological domaini273 – 277CytoplasmicBy similarity5
Transmembranei278 – 298Helical; Name=2By similarityAdd BLAST21
Topological domaini299 – 309ExtracellularBy similarityAdd BLAST11
Transmembranei310 – 330Helical; Name=3By similarityAdd BLAST21
Topological domaini331 – 425CytoplasmicBy similarityAdd BLAST95
Transmembranei426 – 446Helical; Name=4By similarityAdd BLAST21
Topological domaini447 – 457ExtracellularBy similarityAdd BLAST11

Keywords - Cellular componenti

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

Pathology & Biotechi

Involvement in diseasei

Defects in Glra1 are the cause of the spasmodic (spd) phenotype, a mouse mutant which resembles the human neurological disease, hyperekplexia (or startle disease (STHE)) (PubMed:7920629). Defects in Glra1 are the cause of the lethal oscillator (spd-ot) phenotype. Mutant mice display a fine motor tremor and muscle spasms that begin at 2 weeks of age and progressively worsen, resulting in death by 3 weeks of age (PubMed:7874121). Heterozygous mice show an increased acoustic startle response (PubMed:9145798). Neurons from homozygous oscillator mice have dramatically reduced amplitude and frequency of glycinergic inhibitory postsynaptic currents (PubMed:16672662). The oscillator phenotype is due to the complete absence of Glra1 protein (PubMed:9145798).4 Publications

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi108D → A: Eliminates potentiation of glycine-mediated currents by Zn(2+) and causes neuromotor defects similar to human startle disease. 1 Publication1
Mutagenesisi421 – 422KK → AA: Reduces the increase of channel activity in response to ethanol and improves tolerance of intoxicating levels of alcohol. 1 Publication2

Keywords - Diseasei

Disease mutation

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 28By similarityAdd BLAST28
ChainiPRO_000000041329 – 457Glycine receptor subunit alpha-1Add BLAST429

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi66N-linked (GlcNAc...) asparagineCurated1
Disulfide bondi166 ↔ 180By similarity
Disulfide bondi226 ↔ 237By similarity

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

PaxDbiQ64018
PRIDEiQ64018

PTM databases

iPTMnetiQ64018
PhosphoSitePlusiQ64018
SwissPalmiQ64018

Expressioni

Tissue specificityi

Detected in spinal cord neurons (PubMed:9145798, PubMed:17114051, PubMed:24801766). Detected in brain stem neurons (PubMed:16672662, PubMed:24801766). Detected at lower levels in hippocampus and cerebellum (PubMed:24801766). Detected in the inner plexiform layer of the retina (at protein level) (PubMed:12975813).5 Publications

Gene expression databases

BgeeiENSMUSG00000000263
CleanExiMM_GLRA1
ExpressionAtlasiQ64018 baseline and differential
GenevisibleiQ64018 MM

Interactioni

Subunit structurei

Homopentamer (in vitro). Interacts with GLRB to form heteropentameric channels; this is probably the predominant form in vivo. Heteropentamer composed of two GLRA1 and three GLRB. Heteropentamer composed of three GLRA1 and two GLRB. Both homopentamers and heteropentamers form functional ion channels, but their characteristics are subtly different.By similarity

GO - Molecular functioni

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000099777

Structurei

3D structure databases

ProteinModelPortaliQ64018
SMRiQ64018
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni230 – 235Strychnine-bindingBy similarity6

Domaini

The channel pore is formed by pentameric assembly of the second transmembrane domain from all five subunits. Channel opening is effected by an outward rotation of the transmembrane domains that increases the diameter of the pore.By similarity

Sequence similaritiesi

Keywords - Domaini

Signal, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG3643 Eukaryota
ENOG410XPWH LUCA
GeneTreeiENSGT00760000118821
HOGENOMiHOG000231336
HOVERGENiHBG051707
InParanoidiQ64018
KOiK05193
OMAiFNFAYGM
OrthoDBiEOG091G0805
PhylomeDBiQ64018
TreeFamiTF315453

Family and domain databases

Gene3Di2.70.170.10, 1 hit
InterProiView protein in InterPro
IPR006028 GABAA/Glycine_rcpt
IPR008127 Glycine_rcpt_A
IPR008128 Glycine_rcpt_A1
IPR006202 Neur_chan_lig-bd
IPR036734 Neur_chan_lig-bd_sf
IPR006201 Neur_channel
IPR036719 Neuro-gated_channel_TM_sf
IPR006029 Neurotrans-gated_channel_TM
IPR018000 Neurotransmitter_ion_chnl_CS
PANTHERiPTHR18945 PTHR18945, 1 hit
PfamiView protein in Pfam
PF02931 Neur_chan_LBD, 1 hit
PF02932 Neur_chan_memb, 1 hit
PRINTSiPR00253 GABAARECEPTR
PR01673 GLYRALPHA
PR01674 GLYRALPHA1
PR00252 NRIONCHANNEL
SUPFAMiSSF63712 SSF63712, 1 hit
SSF90112 SSF90112, 1 hit
TIGRFAMsiTIGR00860 LIC, 1 hit
PROSITEiView protein in PROSITE
PS00236 NEUROTR_ION_CHANNEL, 1 hit

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 splicing. AlignAdd to basket

Isoform a (identifier: Q64018-1) [UniParc]FASTAAdd to basket
Also known as: Long

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
MYSFNTLRFY LWETIVFFSL AASKEAEAAR SAPKPMSPSD FLDKLMGRTS
60 70 80 90 100
GYDARIRPNF KGPPVNVSCN IFINSFGSIA ETTMDYRVNI FLRQQWNDPR
110 120 130 140 150
LAYNEYPDDS LDLDPSMLDS IWKPDLFFAN EKGAHFHEIT TDNKLLRISR
160 170 180 190 200
NGNVLYSIRI TLTLACPMDL KNFPMDVQTC IMQLESFGYT MNDLIFEWQE
210 220 230 240 250
QGAVQVADGL TLPQFILKEE KDLRYCTKHY NTGKFTCIEA RFHLERQMGY
260 270 280 290 300
YLIQMYIPSL LIVILSWISF WINMDAAPAR VGLGITTVLT MTTQSSGSRA
310 320 330 340 350
SLPKVSYVKA IDIWMAVCLL FVFSALLEYA AVNFVSRQHK ELLRFRRKRR
360 370 380 390 400
HHKSPMLNLF QDDEGGEGRF NFSAYGMGPA CLQAKDGISV KGANNNNTTN
410 420 430 440 450
PPPAPSKSPE EMRKLFIQRA KKIDKISRIG FPMAFLIFNM FYWIIYKIVR

REDVHNK
Length:457
Mass (Da):52,657
Last modified:December 8, 2000 - v2
Checksum:i29268DC4991A6E20
GO
Isoform b (identifier: Q64018-2) [UniParc]FASTAAdd to basket
Also known as: Short

The sequence of this isoform differs from the canonical sequence as follows:
     354-361: Missing.

Show »
Length:449
Mass (Da):51,726
Checksum:i4EDDAEB87874A153
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti84M → I in CAB52398 (PubMed:7507926).Curated1
Sequence conflicti84M → I in CAB52399 (PubMed:7507926).Curated1
Sequence conflicti426 – 429ISRI → NISH in CAB52398 (PubMed:7507926).Curated4
Sequence conflicti426 – 429ISRI → NISH in CAB52399 (PubMed:7507926).Curated4

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural varianti80A → S in spd. 1 Publication1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_000080354 – 361Missing in isoform b. 1 Publication8

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
S73717 mRNA Translation: AAB32157.2
S73718 mRNA Translation: AAB32158.2
X75832
, X75833, X75834, X75835, X75836, X75837, X75838, X75839, X75840 Genomic DNA Translation: CAB52398.1
X75832
, X75833, X75834, X75835, X75836, X75837, X75838, X75839, X75840 Genomic DNA Translation: CAB52399.1
AL596207 Genomic DNA Translation: CAI35359.1
CCDSiCCDS24715.1 [Q64018-2]
CCDS70190.1 [Q64018-1]
PIRiC49970
RefSeqiNP_001277750.1, NM_001290821.1 [Q64018-1]
UniGeneiMm.89320

Genome annotation databases

EnsembliENSMUST00000075603; ENSMUSP00000075032; ENSMUSG00000000263 [Q64018-1]
ENSMUST00000102716; ENSMUSP00000099777; ENSMUSG00000000263 [Q64018-2]
GeneIDi14654
KEGGimmu:14654
UCSCiuc007izo.2 mouse [Q64018-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiGLRA1_MOUSE
AccessioniPrimary (citable) accession number: Q64018
Secondary accession number(s): Q5NCT8
, Q64019, Q9R0Y6, Q9R0Y7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: December 8, 2000
Last modified: July 18, 2018
This is version 165 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. SIMILARITY comments
    Index of protein domains and families

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