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Protein

Homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein

Gene

HERPUD1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Component of the endoplasmic reticulum quality control (ERQC) system also called ER-associated degradation (ERAD) involved in ubiquitin-dependent degradation of misfolded endoplasmic reticulum proteins. Could enhance presenilin-mediated amyloid-beta protein 40 generation. Binds to ubiquilins and this interaction is required for efficient degradation of CD3D via the ERAD pathway (PubMed:18307982).2 Publications

Miscellaneous

Although the precise topology is not known, experimental data suggest that both the N- and C-termini face the cytosol.

GO - Molecular functioni

  • ion channel binding Source: ParkinsonsUK-UCL

GO - Biological processi

Keywordsi

Biological processUnfolded protein response

Enzyme and pathway databases

ReactomeiR-HSA-380994 ATF4 activates genes
SIGNORiQ15011

Names & Taxonomyi

Protein namesi
Recommended name:
Homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein
Alternative name(s):
Methyl methanesulfonate (MMF)-inducible fragment protein 1
Gene namesi
Name:HERPUD1
Synonyms:HERP, KIAA0025, MIF1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 16

Organism-specific databases

EuPathDBiHostDB:ENSG00000051108.14
HGNCiHGNC:13744 HERPUD1
MIMi608070 gene
neXtProtiNX_Q15011

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 domaini1 – 263CytoplasmicSequence analysisAdd BLAST263
Transmembranei264 – 284HelicalSequence analysisAdd BLAST21
Topological domaini285 – 289LumenalSequence analysis5
Transmembranei290 – 310HelicalSequence analysisAdd BLAST21
Topological domaini311 – 391CytoplasmicSequence analysisAdd BLAST81

Keywords - Cellular componenti

Endoplasmic reticulum, Membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi9709
OpenTargetsiENSG00000051108
PharmGKBiPA29252

Polymorphism and mutation databases

DMDMi3123034

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001149201 – 391Homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 proteinAdd BLAST391

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei1N-acetylmethionineCombined sources1
Modified residuei135PhosphoserineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiQ15011
MaxQBiQ15011
PaxDbiQ15011
PeptideAtlasiQ15011
PRIDEiQ15011

PTM databases

iPTMnetiQ15011
PhosphoSitePlusiQ15011

Expressioni

Tissue specificityi

Widely expressed; in the brain, expression seems to be restricted to neurons and vascular smooth muscle cells. Present in activated microglia in senile plaques in the brain of patients with Alzheimer disease.

Inductioni

Up-regulated by endoplasmic reticulum stress and CREB3.2 Publications

Gene expression databases

BgeeiENSG00000051108
CleanExiHS_HERPUD1
ExpressionAtlasiQ15011 baseline and differential
GenevisibleiQ15011 HS

Organism-specific databases

HPAiCAB037041
CAB037104
HPA040754
HPA041219

Interactioni

Subunit structurei

Interacts with PSEN1 and PSEN2. Interacts with SYVN1 and UBXN6. Interacts with UBQLN1, UBQLN2 and UBQLN4.4 Publications

GO - Molecular functioni

  • ion channel binding Source: ParkinsonsUK-UCL

Protein-protein interaction databases

BioGridi115060, 32 interactors
DIPiDIP-46662N
IntActiQ15011, 11 interactors
MINTiQ15011
STRINGi9606.ENSP00000409555

Structurei

Secondary structure

1391
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi11 – 15Combined sources5
Beta strandi17 – 20Combined sources4
Beta strandi24 – 27Combined sources4
Helixi34 – 44Combined sources11
Turni51 – 53Combined sources3
Beta strandi55 – 58Combined sources4
Beta strandi65 – 67Combined sources3
Helixi69 – 72Combined sources4
Beta strandi75 – 77Combined sources3
Beta strandi79 – 85Combined sources7

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1WGDNMR-A10-90[»]
ProteinModelPortaliQ15011
SMRiQ15011
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ15011

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini10 – 72Ubiquitin-likePROSITE-ProRule annotationAdd BLAST63

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni115 – 200Interaction with UBQLN11 PublicationAdd BLAST86

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG4583 Eukaryota
ENOG410ZJAF LUCA
GeneTreeiENSGT00390000017671
HOGENOMiHOG000252989
HOVERGENiHBG051899
InParanoidiQ15011
KOiK14027
OMAiANQNMRM
OrthoDBiEOG091G0P9J
PhylomeDBiQ15011
TreeFamiTF324319

Family and domain databases

InterProiView protein in InterPro
IPR029071 Ubiquitin-like_domsf
IPR000626 Ubiquitin_dom
PfamiView protein in Pfam
PF00240 ubiquitin, 1 hit
SMARTiView protein in SMART
SM00213 UBQ, 1 hit
SUPFAMiSSF54236 SSF54236, 1 hit
PROSITEiView protein in PROSITE
PS50053 UBIQUITIN_2, 1 hit

Sequences (4)i

Sequence statusi: Complete.

This entry describes 4 isoformsi produced by alternative splicing. AlignAdd to basket

Note: Experimental confirmation may be lacking for some isoforms.
Isoform 1 (identifier: Q15011-1) [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
MESETEPEPV TLLVKSPNQR HRDLELSGDR GWSVGHLKAH LSRVYPERPR
60 70 80 90 100
PEDQRLIYSG KLLLDHQCLR DLLPKQEKRH VLHLVCNVKS PSKMPEINAK
110 120 130 140 150
VAESTEEPAG SNRGQYPEDS SSDGLRQREV LRNLSSPGWE NISRPEAAQQ
160 170 180 190 200
AFQGLGPGFS GYTPYGWLQL SWFQQIYARQ YYMQYLAATA ASGAFVPPPS
210 220 230 240 250
AQEIPVVSAP APAPIHNQFP AENQPANQNA APQVVVNPGA NQNLRMNAQG
260 270 280 290 300
GPIVEEDDEI NRDWLDWTYS AATFSVFLSI LYFYSSLSRF LMVMGATVVM
310 320 330 340 350
YLHHVGWFPF RPRPVQNFPN DGPPPDVVNQ DPNNNLQEGT DPETEDPNHL
360 370 380 390
PPDRDVLDGE QTSPSFMSTA WLVFKTFFAS LLPEGPPAIA N
Length:391
Mass (Da):43,720
Last modified:November 1, 1996 - v1
Checksum:i3CA827DC7EF0ED22
GO
Isoform 2 (identifier: Q15011-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     76-76: Missing.

Show »
Length:390
Mass (Da):43,592
Checksum:i7BCA8854403C71AF
GO
Isoform 3 (identifier: Q15011-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     76-76: Missing.
     145-302: Missing.

Show »
Length:232
Mass (Da):26,220
Checksum:iBE916EB3E3CD79C0
GO
Isoform 4 (identifier: Q15011-4) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     75-99: Missing.

Note: No experimental confirmation available.
Show »
Length:366
Mass (Da):40,851
Checksum:iDD39A053E4D46626
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_02427750R → H. Corresponds to variant dbSNP:rs2217332Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_04733375 – 99Missing in isoform 4. CuratedAdd BLAST25
Alternative sequenceiVSP_00670876Missing in isoform 2 and isoform 3. 2 Publications1
Alternative sequenceiVSP_006709145 – 302Missing in isoform 3. 1 PublicationAdd BLAST158

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB034989 mRNA Translation: BAB07891.1
D14695 mRNA Translation: BAA03521.1
AF055001 mRNA Translation: AAC09355.1
AF055003 mRNA Translation: AAC09357.1
AB034990 Genomic DNA Translation: BAB19010.1
CR457116 mRNA Translation: CAG33397.1
AC012181 Genomic DNA No translation available.
BC000086 mRNA Translation: AAH00086.1
BC008320 mRNA Translation: AAH08320.1
BC009739 mRNA Translation: AAH09739.1
BC032673 mRNA Translation: AAH32673.1
CCDSiCCDS10771.1 [Q15011-1]
CCDS45492.1 [Q15011-2]
RefSeqiNP_001010989.1, NM_001010989.2 [Q15011-2]
NP_001259032.1, NM_001272103.1
NP_055500.1, NM_014685.3 [Q15011-1]
UniGeneiHs.146393

Genome annotation databases

EnsembliENST00000300302; ENSP00000300302; ENSG00000051108 [Q15011-2]
ENST00000344114; ENSP00000340931; ENSG00000051108 [Q15011-3]
ENST00000379792; ENSP00000369118; ENSG00000051108 [Q15011-4]
ENST00000439977; ENSP00000409555; ENSG00000051108 [Q15011-1]
GeneIDi9709
KEGGihsa:9709
UCSCiuc002eke.3 human [Q15011-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Entry informationi

Entry nameiHERP1_HUMAN
AccessioniPrimary (citable) accession number: Q15011
Secondary accession number(s): E9PGD1
, O60644, Q6IAN8, Q96D92
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: November 1, 1996
Last modified: April 25, 2018
This is version 169 of the entry and version 1 of the sequence. See 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

3D-structure, Complete proteome, Reference proteome
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Main funding by: National Institutes of Health