S61A1_DANRE
ID S61A1_DANRE Reviewed; 476 AA.
AC Q90ZM2; Q7ZU31;
DT 07-JUN-2004, integrated into UniProtKB/Swiss-Prot.
DT 23-JAN-2007, sequence version 3.
DT 03-AUG-2022, entry version 123.
DE RecName: Full=Protein transport protein Sec61 subunit alpha-like 1;
GN Name=sec61al1; Synonyms=sec61a, sec61aa;
OS Danio rerio (Zebrafish) (Brachydanio rerio).
OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi;
OC Actinopterygii; Neopterygii; Teleostei; Ostariophysi; Cypriniformes;
OC Danionidae; Danioninae; Danio.
OX NCBI_TaxID=7955;
RN [1]
RP NUCLEOTIDE SEQUENCE [MRNA].
RA Hartmann E.;
RL Submitted (APR-2001) to the EMBL/GenBank/DDBJ databases.
RN [2]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RC STRAIN=AB; TISSUE=Kidney;
RG NIH - Zebrafish Gene Collection (ZGC) project;
RL Submitted (JAN-2003) to the EMBL/GenBank/DDBJ databases.
RN [3]
RP FUNCTION.
RX PubMed=27392076; DOI=10.1016/j.ajhg.2016.05.028;
RA Bolar N.A., Golzio C., Zivna M., Hayot G., Van Hemelrijk C., Schepers D.,
RA Vandeweyer G., Hoischen A., Huyghe J.R., Raes A., Matthys E., Sys E.,
RA Azou M., Gubler M.C., Praet M., Van Camp G., McFadden K., Pediaditakis I.,
RA Pristoupilova A., Hodanova K., Vyletal P., Hartmannova H., Stranecky V.,
RA Hulkova H., Baresova V., Jedlickova I., Sovova J., Hnizda A., Kidd K.,
RA Bleyer A.J., Spong R.S., Vande Walle J., Mortier G., Brunner H.,
RA Van Laer L., Kmoch S., Katsanis N., Loeys B.L.;
RT "Heterozygous loss-of-function SEC61A1 mutations cause autosomal-dominant
RT tubulo-interstitial and glomerulocystic kidney disease with anemia.";
RL Am. J. Hum. Genet. 99:174-187(2016).
CC -!- FUNCTION: Appears to play a crucial role in the insertion of secretory
CC and membrane polypeptides into the ER. It is required for assembly of
CC membrane and secretory proteins. Found to be tightly associated with
CC membrane-bound ribosomes, either directly or through adapter proteins
CC (By similarity). Necessary for the biogenesis and transport of multi-
CC pass membrane proteins into the ER membrane (By similarity). Plays a
CC role in the pronephric kidney tubule development (PubMed:27392076).
CC {ECO:0000250|UniProtKB:P61619, ECO:0000269|PubMed:27392076}.
CC -!- SUBUNIT: Heterotrimeric complex composed of SEC61-alpha, SEC61-beta and
CC SEC61-gamma (By similarity). Component of the ribosome-associated ER
CC translocon complex (By similarity). {ECO:0000250|UniProtKB:P38377,
CC ECO:0000250|UniProtKB:P61619}.
CC -!- SUBCELLULAR LOCATION: Endoplasmic reticulum membrane
CC {ECO:0000250|UniProtKB:P61619}; Multi-pass membrane protein
CC {ECO:0000305}. Note=Localizes exclusively in granular structures in the
CC endoplasmic reticulum (ER). {ECO:0000250|UniProtKB:P61619}.
CC -!- SIMILARITY: Belongs to the SecY/SEC61-alpha family. {ECO:0000305}.
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DR EMBL; AY029527; AAK40295.1; -; mRNA.
DR EMBL; BC044351; AAH44351.1; -; mRNA.
DR EMBL; BC066715; AAH66715.1; -; mRNA.
DR RefSeq; NP_705945.1; NM_153659.1.
DR AlphaFoldDB; Q90ZM2; -.
DR SMR; Q90ZM2; -.
DR STRING; 7955.ENSDARP00000033318; -.
DR PaxDb; Q90ZM2; -.
DR Ensembl; ENSDART00000034730; ENSDARP00000033318; ENSDARG00000021669.
DR GeneID; 192298; -.
DR KEGG; dre:192298; -.
DR CTD; 29927; -.
DR ZFIN; ZDB-GENE-020418-2; sec61a1.
DR eggNOG; KOG1373; Eukaryota.
DR GeneTree; ENSGT00390000003721; -.
DR HOGENOM; CLU_031763_2_0_1; -.
DR InParanoid; Q90ZM2; -.
DR OMA; VIEDPMH; -.
DR OrthoDB; 482911at2759; -.
DR PhylomeDB; Q90ZM2; -.
DR TreeFam; TF300348; -.
DR PRO; PR:Q90ZM2; -.
DR Proteomes; UP000000437; Genome assembly.
DR Proteomes; UP000814640; Chromosome 6.
DR Bgee; ENSDARG00000021669; Expressed in intestine and 41 other tissues.
DR GO; GO:0005789; C:endoplasmic reticulum membrane; ISS:UniProtKB.
DR GO; GO:0030176; C:integral component of endoplasmic reticulum membrane; ISS:UniProtKB.
DR GO; GO:0005784; C:Sec61 translocon complex; IBA:GO_Central.
DR GO; GO:0008320; F:protein transmembrane transporter activity; IBA:GO_Central.
DR GO; GO:0043022; F:ribosome binding; ISS:UniProtKB.
DR GO; GO:0005048; F:signal sequence binding; IBA:GO_Central.
DR GO; GO:0006613; P:cotranslational protein targeting to membrane; ISS:UniProtKB.
DR GO; GO:0021986; P:habenula development; IMP:ZFIN.
DR GO; GO:0031204; P:post-translational protein targeting to membrane, translocation; IBA:GO_Central.
DR GO; GO:0039019; P:pronephric nephron development; IMP:UniProtKB.
DR GO; GO:0045048; P:protein insertion into ER membrane; ISS:UniProtKB.
DR GO; GO:0006616; P:SRP-dependent cotranslational protein targeting to membrane, translocation; IBA:GO_Central.
DR Gene3D; 1.10.3370.10; -; 1.
DR InterPro; IPR002208; SecY/SEC61-alpha.
DR InterPro; IPR030659; SecY_CS.
DR InterPro; IPR023201; SecY_dom_sf.
DR InterPro; IPR019561; Translocon_Sec61/SecY_plug_dom.
DR PANTHER; PTHR10906; PTHR10906; 1.
DR Pfam; PF10559; Plug_translocon; 1.
DR Pfam; PF00344; SecY; 1.
DR PIRSF; PIRSF004557; SecY; 1.
DR SUPFAM; SSF103491; SSF103491; 1.
DR TIGRFAMs; TIGR00967; 3a0501s007; 1.
DR PROSITE; PS00755; SECY_1; 1.
DR PROSITE; PS00756; SECY_2; 1.
PE 2: Evidence at transcript level;
KW Developmental protein; Endoplasmic reticulum; Membrane; Protein transport;
KW Reference proteome; Translocation; Transmembrane; Transmembrane helix;
KW Transport.
FT INIT_MET 1
FT /note="Removed"
FT /evidence="ECO:0000250"
FT CHAIN 2..476
FT /note="Protein transport protein Sec61 subunit alpha-like
FT 1"
FT /id="PRO_0000131797"
FT TOPO_DOM 2..33
FT /note="Cytoplasmic"
FT /evidence="ECO:0000255"
FT TRANSMEM 34..53
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TOPO_DOM 54..76
FT /note="Lumenal"
FT /evidence="ECO:0000255"
FT TRANSMEM 77..96
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TOPO_DOM 97..117
FT /note="Cytoplasmic"
FT /evidence="ECO:0000255"
FT TRANSMEM 118..138
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TOPO_DOM 139..144
FT /note="Lumenal"
FT /evidence="ECO:0000255"
FT TRANSMEM 145..165
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TOPO_DOM 166..172
FT /note="Cytoplasmic"
FT /evidence="ECO:0000255"
FT TRANSMEM 173..193
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TOPO_DOM 194..240
FT /note="Lumenal"
FT /evidence="ECO:0000255"
FT TRANSMEM 241..261
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TOPO_DOM 262..288
FT /note="Cytoplasmic"
FT /evidence="ECO:0000255"
FT TRANSMEM 289..309
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TOPO_DOM 310..354
FT /note="Lumenal"
FT /evidence="ECO:0000255"
FT TRANSMEM 355..375
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TOPO_DOM 376..420
FT /note="Cytoplasmic"
FT /evidence="ECO:0000255"
FT TRANSMEM 421..441
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TOPO_DOM 442..445
FT /note="Lumenal"
FT /evidence="ECO:0000255"
FT TRANSMEM 446..462
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TOPO_DOM 463..476
FT /note="Cytoplasmic"
FT /evidence="ECO:0000255"
FT CONFLICT 312
FT /note="F -> S (in Ref. 1; AAK40295)"
FT /evidence="ECO:0000305"
SQ SEQUENCE 476 AA; 52298 MW; 65CA78CE973E09AD CRC64;
MAIKFLEVIK PFCAVLPEIQ KPERKIQFRE KVLWTAITLF IFLVCCQIPL FGIMSSDSAD
PFYWMRVILA SNRGTLMELG ISPIVTSGLI MQLLAGAKII EVGDTPKDRA LFNGAQKLFG
MIITIGQAIV YVMTGMYGDP SEMGAGICLL IIIQLFVAGL IVLLLDELLQ KGYGLGSGIS
LFIATNICET IVWKAFSPTT VNTGRGTEFE GAIIALFHLL ATRTDKVRAL REAFYRQNLP
NLMNLIATVF VFAVVIYFQG FRVDLPIKSA RYRGQYNTYP IKLFYTSNIP IILQSALVSN
LYVISQMLST RFSGNFLVNL LGTWSDTSSG GPARAYPVGG LCYYLSPPES FGSVLDDPVH
AVIYIVFMLG SCAFFSKTWI EVSGSSAKDV AKQLKEQQMV MRGHRETSMV HELNRYIPTA
AAFGGLCIGG LSVMADFLGA IGSGTGILLA VTIIYQYFEI FVKEQSEVGS MGALLF
