GRPE1_HUMAN
ID GRPE1_HUMAN Reviewed; 217 AA.
AC Q9HAV7; B2R783; Q549M6;
DT 11-JUL-2001, integrated into UniProtKB/Swiss-Prot.
DT 11-JUL-2001, sequence version 2.
DT 03-AUG-2022, entry version 178.
DE RecName: Full=GrpE protein homolog 1, mitochondrial;
DE AltName: Full=HMGE;
DE AltName: Full=Mt-GrpE#1;
DE Flags: Precursor;
GN Name=GRPEL1; Synonyms=GREPEL1;
OS Homo sapiens (Human).
OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia;
OC Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae;
OC Homo.
OX NCBI_TaxID=9606;
RN [1]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RC TISSUE=Uterus;
RX PubMed=14702039; DOI=10.1038/ng1285;
RA Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R.,
RA Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H.,
RA Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S.,
RA Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K.,
RA Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H.,
RA Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M.,
RA Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K.,
RA Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T.,
RA Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M.,
RA Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S.,
RA Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H.,
RA Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K.,
RA Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N.,
RA Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S.,
RA Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O.,
RA Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H.,
RA Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B.,
RA Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y.,
RA Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K.,
RA Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T.,
RA Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T.,
RA Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y.,
RA Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H.,
RA Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y.,
RA Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H.,
RA Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O.,
RA Isogai T., Sugano S.;
RT "Complete sequencing and characterization of 21,243 full-length human
RT cDNAs.";
RL Nat. Genet. 36:40-45(2004).
RN [2]
RP NUCLEOTIDE SEQUENCE [MRNA] OF 2-117, CHARACTERIZATION, FUNCTION,
RP SUBCELLULAR LOCATION, AND INTERACTION WITH HSP70; HSC70 AND HSJ1B.
RC TISSUE=Brain;
RX PubMed=11311562; DOI=10.1016/s0378-1119(01)00396-1;
RA Choglay A.A., Chapple J.P., Blatch G.L., Cheetham M.E.;
RT "Identification and characterization of a human mitochondrial homologue of
RT the bacterial co-chaperone GrpE.";
RL Gene 267:125-134(2001).
RN [3]
RP NUCLEOTIDE SEQUENCE [MRNA].
RA Wan Y.Z., Yu L., Zhang H.L., Fu Q., Jiang J.X., Zhao S.Y.;
RT "Cloning and characterization of a novel human cDNA homology to
RT R.norvegicus stress-inducible chaperone mt-GrpE#1 mRNA.";
RL Submitted (JUL-2003) to the EMBL/GenBank/DDBJ databases.
RN [4]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RC TISSUE=Placenta;
RA Li W.B., Gruber C., Jessee J., Polayes D.;
RT "Full-length cDNA libraries and normalization.";
RL Submitted (FEB-2001) to the EMBL/GenBank/DDBJ databases.
RN [5]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
RA Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M.,
RA Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J.,
RA Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S.,
RA Turner R., Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H.,
RA Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K.,
RA Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D.,
RA Hunkapiller M.W., Myers E.W., Venter J.C.;
RL Submitted (SEP-2005) to the EMBL/GenBank/DDBJ databases.
RN [6]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RC TISSUE=Testis;
RX PubMed=15489334; DOI=10.1101/gr.2596504;
RG The MGC Project Team;
RT "The status, quality, and expansion of the NIH full-length cDNA project:
RT the Mammalian Gene Collection (MGC).";
RL Genome Res. 14:2121-2127(2004).
RN [7]
RP ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-215, AND IDENTIFICATION BY MASS
RP SPECTROMETRY [LARGE SCALE ANALYSIS].
RX PubMed=19608861; DOI=10.1126/science.1175371;
RA Choudhary C., Kumar C., Gnad F., Nielsen M.L., Rehman M., Walther T.C.,
RA Olsen J.V., Mann M.;
RT "Lysine acetylation targets protein complexes and co-regulates major
RT cellular functions.";
RL Science 325:834-840(2009).
RN [8]
RP IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
RX PubMed=21269460; DOI=10.1186/1752-0509-5-17;
RA Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T.,
RA Bennett K.L., Superti-Furga G., Colinge J.;
RT "Initial characterization of the human central proteome.";
RL BMC Syst. Biol. 5:17-17(2011).
RN [9]
RP IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
RC TISSUE=Liver;
RX PubMed=24275569; DOI=10.1016/j.jprot.2013.11.014;
RA Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D., Wang L.,
RA Ye M., Zou H.;
RT "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver
RT phosphoproteome.";
RL J. Proteomics 96:253-262(2014).
RN [10]
RP IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
RX PubMed=25944712; DOI=10.1002/pmic.201400617;
RA Vaca Jacome A.S., Rabilloud T., Schaeffer-Reiss C., Rompais M., Ayoub D.,
RA Lane L., Bairoch A., Van Dorsselaer A., Carapito C.;
RT "N-terminome analysis of the human mitochondrial proteome.";
RL Proteomics 15:2519-2524(2015).
CC -!- FUNCTION: Essential component of the PAM complex, a complex required
CC for the translocation of transit peptide-containing proteins from the
CC inner membrane into the mitochondrial matrix in an ATP-dependent manner
CC (By similarity). Seems to control the nucleotide-dependent binding of
CC mitochondrial HSP70 to substrate proteins (PubMed:11311562).
CC {ECO:0000250|UniProtKB:P38523, ECO:0000269|PubMed:11311562}.
CC -!- SUBUNIT: Probable component of the PAM complex at least composed of a
CC mitochondrial HSP70 protein, GRPEL1 or GRPEL2, TIMM44, TIMM16/PAM16 and
CC TIMM14/DNAJC19 (By similarity). Binds to HSP70, HSC70 and HSJ1B
CC (PubMed:11311562). {ECO:0000250|UniProtKB:P38523,
CC ECO:0000269|PubMed:11311562}.
CC -!- INTERACTION:
CC Q9HAV7; Q2TAC2-2: CCDC57; NbExp=3; IntAct=EBI-1043499, EBI-10961624;
CC Q9HAV7; P07902: GALT; NbExp=3; IntAct=EBI-1043499, EBI-750827;
CC Q9HAV7; Q9HAV7: GRPEL1; NbExp=5; IntAct=EBI-1043499, EBI-1043499;
CC Q9HAV7; P38646: HSPA9; NbExp=2; IntAct=EBI-1043499, EBI-354932;
CC Q9HAV7; O15160: POLR1C; NbExp=4; IntAct=EBI-1043499, EBI-1055079;
CC Q9HAV7; Q9NZD8: SPG21; NbExp=3; IntAct=EBI-1043499, EBI-742688;
CC -!- SUBCELLULAR LOCATION: Mitochondrion matrix
CC {ECO:0000269|PubMed:11311562}.
CC -!- SIMILARITY: Belongs to the GrpE family. {ECO:0000305}.
CC ---------------------------------------------------------------------------
CC Copyrighted by the UniProt Consortium, see https://www.uniprot.org/terms
CC Distributed under the Creative Commons Attribution (CC BY 4.0) License
CC ---------------------------------------------------------------------------
DR EMBL; AK312882; BAG35730.1; -; mRNA.
DR EMBL; AF298592; AAG31605.1; -; mRNA.
DR EMBL; AF087896; AAP97195.1; -; mRNA.
DR EMBL; AL542571; -; NOT_ANNOTATED_CDS; mRNA.
DR EMBL; CH471131; EAW82371.1; -; Genomic_DNA.
DR EMBL; BC024242; AAH24242.1; -; mRNA.
DR CCDS; CCDS3396.1; -.
DR RefSeq; NP_079472.1; NM_025196.3.
DR AlphaFoldDB; Q9HAV7; -.
DR SMR; Q9HAV7; -.
DR BioGRID; 123210; 210.
DR ComplexPortal; CPX-6129; TIM23 mitochondrial inner membrane pre-sequence translocase complex, TIM17A variant.
DR ComplexPortal; CPX-6130; TIM23 mitochondrial inner membrane pre-sequence translocase complex, TIM17B variant.
DR CORUM; Q9HAV7; -.
DR IntAct; Q9HAV7; 73.
DR MINT; Q9HAV7; -.
DR STRING; 9606.ENSP00000264954; -.
DR GlyGen; Q9HAV7; 1 site, 1 O-linked glycan (1 site).
DR iPTMnet; Q9HAV7; -.
DR MetOSite; Q9HAV7; -.
DR PhosphoSitePlus; Q9HAV7; -.
DR SwissPalm; Q9HAV7; -.
DR BioMuta; GRPEL1; -.
DR DMDM; 18202951; -.
DR EPD; Q9HAV7; -.
DR jPOST; Q9HAV7; -.
DR MassIVE; Q9HAV7; -.
DR MaxQB; Q9HAV7; -.
DR PaxDb; Q9HAV7; -.
DR PeptideAtlas; Q9HAV7; -.
DR PRIDE; Q9HAV7; -.
DR ProteomicsDB; 81449; -.
DR TopDownProteomics; Q9HAV7; -.
DR Antibodypedia; 22707; 155 antibodies from 24 providers.
DR DNASU; 80273; -.
DR Ensembl; ENST00000264954.5; ENSP00000264954.4; ENSG00000109519.13.
DR GeneID; 80273; -.
DR KEGG; hsa:80273; -.
DR MANE-Select; ENST00000264954.5; ENSP00000264954.4; NM_025196.4; NP_079472.1.
DR UCSC; uc003gjy.2; human.
DR CTD; 80273; -.
DR DisGeNET; 80273; -.
DR GeneCards; GRPEL1; -.
DR HGNC; HGNC:19696; GRPEL1.
DR HPA; ENSG00000109519; Low tissue specificity.
DR MIM; 606173; gene.
DR neXtProt; NX_Q9HAV7; -.
DR OpenTargets; ENSG00000109519; -.
DR PharmGKB; PA134918140; -.
DR VEuPathDB; HostDB:ENSG00000109519; -.
DR eggNOG; KOG3003; Eukaryota.
DR GeneTree; ENSGT00390000005589; -.
DR HOGENOM; CLU_057217_0_1_1; -.
DR InParanoid; Q9HAV7; -.
DR OMA; YAYEKIA; -.
DR OrthoDB; 1525208at2759; -.
DR PhylomeDB; Q9HAV7; -.
DR TreeFam; TF105284; -.
DR PathwayCommons; Q9HAV7; -.
DR Reactome; R-HSA-1268020; Mitochondrial protein import.
DR SignaLink; Q9HAV7; -.
DR SIGNOR; Q9HAV7; -.
DR BioGRID-ORCS; 80273; 778 hits in 1086 CRISPR screens.
DR ChiTaRS; GRPEL1; human.
DR GenomeRNAi; 80273; -.
DR Pharos; Q9HAV7; Tbio.
DR PRO; PR:Q9HAV7; -.
DR Proteomes; UP000005640; Chromosome 4.
DR RNAct; Q9HAV7; protein.
DR Bgee; ENSG00000109519; Expressed in left adrenal gland and 191 other tissues.
DR Genevisible; Q9HAV7; HS.
DR GO; GO:0005743; C:mitochondrial inner membrane; IC:ComplexPortal.
DR GO; GO:0005759; C:mitochondrial matrix; IDA:UniProtKB.
DR GO; GO:0005739; C:mitochondrion; IDA:HPA.
DR GO; GO:0005654; C:nucleoplasm; IDA:HPA.
DR GO; GO:0001405; C:PAM complex, Tim23 associated import motor; IBA:GO_Central.
DR GO; GO:0005744; C:TIM23 mitochondrial import inner membrane translocase complex; IC:ComplexPortal.
DR GO; GO:0000774; F:adenyl-nucleotide exchange factor activity; IBA:GO_Central.
DR GO; GO:0051087; F:chaperone binding; IEA:InterPro.
DR GO; GO:0042802; F:identical protein binding; IPI:IntAct.
DR GO; GO:0042803; F:protein homodimerization activity; IEA:InterPro.
DR GO; GO:0051082; F:unfolded protein binding; IDA:UniProtKB.
DR GO; GO:0006886; P:intracellular protein transport; IC:ComplexPortal.
DR GO; GO:0006457; P:protein folding; IEA:InterPro.
DR GO; GO:0030150; P:protein import into mitochondrial matrix; IBA:GO_Central.
DR CDD; cd00446; GrpE; 1.
DR Gene3D; 2.30.22.10; -; 1.
DR Gene3D; 3.90.20.20; -; 1.
DR HAMAP; MF_01151; GrpE; 1.
DR InterPro; IPR000740; GrpE.
DR InterPro; IPR013805; GrpE_coiled_coil.
DR InterPro; IPR009012; GrpE_head.
DR PANTHER; PTHR21237; PTHR21237; 1.
DR Pfam; PF01025; GrpE; 1.
DR PRINTS; PR00773; GRPEPROTEIN.
DR SUPFAM; SSF51064; SSF51064; 1.
DR SUPFAM; SSF58014; SSF58014; 1.
DR PROSITE; PS01071; GRPE; 1.
PE 1: Evidence at protein level;
KW Acetylation; Chaperone; Mitochondrion; Reference proteome; Transit peptide.
FT TRANSIT 1..27
FT /note="Mitochondrion"
FT /evidence="ECO:0000250"
FT CHAIN 28..217
FT /note="GrpE protein homolog 1, mitochondrial"
FT /id="PRO_0000013049"
FT REGION 29..56
FT /note="Disordered"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT COMPBIAS 29..44
FT /note="Polar residues"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT MOD_RES 94
FT /note="N6-acetyllysine; alternate"
FT /evidence="ECO:0000250|UniProtKB:Q99LP6"
FT MOD_RES 94
FT /note="N6-succinyllysine; alternate"
FT /evidence="ECO:0000250|UniProtKB:Q99LP6"
FT MOD_RES 100
FT /note="N6-acetyllysine"
FT /evidence="ECO:0000250|UniProtKB:Q99LP6"
FT MOD_RES 120
FT /note="N6-succinyllysine"
FT /evidence="ECO:0000250|UniProtKB:Q99LP6"
FT MOD_RES 215
FT /note="N6-acetyllysine; alternate"
FT /evidence="ECO:0007744|PubMed:19608861"
FT MOD_RES 215
FT /note="N6-succinyllysine; alternate"
FT /evidence="ECO:0000250|UniProtKB:Q99LP6"
SQ SEQUENCE 217 AA; 24279 MW; 40499AA3FC7E689E CRC64;
MAAQCVRLAR RSLPALALSL RPSPRLLCTA TKQKNSGQNL EEDMGQSEQK ADPPATEKTL
LEEKVKLEEQ LKETVEKYKR ALADTENLRQ RSQKLVEEAK LYGIQAFCKD LLEVADVLEK
ATQCVPKEEI KDDNPHLKNL YEGLVMTEVQ IQKVFTKHGL LKLNPVGAKF DPYEHEALFH
TPVEGKEPGT VALVSKVGYK LHGRTLRPAL VGVVKEA
