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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: Annotation score: 5 out of 5-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 beta-amyloid protein 40 generation. Binds to ubiquilins and this interaction is required for efficient degradation of CD3D via the ERAD pathway (PubMed:18307982).2 Publications

GO - Molecular functioni

  • ion channel binding Source: ParkinsonsUK-UCL

GO - Biological processi

  • endoplasmic reticulum calcium ion homeostasis Source: ParkinsonsUK-UCL
  • negative regulation of cysteine-type endopeptidase activity involved in apoptotic process Source: Ensembl
  • negative regulation of endoplasmic reticulum stress-induced intrinsic apoptotic signaling pathway Source: ParkinsonsUK-UCL
  • negative regulation of intrinsic apoptotic signaling pathway Source: ParkinsonsUK-UCL
  • PERK-mediated unfolded protein response Source: Reactome
  • positive regulation of ER-associated ubiquitin-dependent protein catabolic process Source: UniProtKB
  • positive regulation of protein binding Source: Ensembl
  • regulation of protein ubiquitination Source: Ensembl
  • response to endoplasmic reticulum stress Source: ParkinsonsUK-UCL
  • response to unfolded protein Source: UniProtKB
  • retrograde protein transport, ER to cytosol Source: ParkinsonsUK-UCL
  • ubiquitin-dependent protein catabolic process Source: Ensembl
Complete GO annotation...

Keywords - Biological processi

Unfolded protein response

Enzyme and pathway databases

BioCyciZFISH:ENSG00000051108-MONOMER.
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

HGNCiHGNC:13744. HERPUD1.

Subcellular locationi

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

GO - Cellular componenti

  • calcium channel complex Source: Ensembl
  • endoplasmic reticulum Source: ParkinsonsUK-UCL
  • endoplasmic reticulum membrane Source: UniProtKB
  • Lewy body core Source: ParkinsonsUK-UCL
  • membrane Source: UniProtKB
Complete GO annotation...

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. 29 interactors.
DIPiDIP-46662N.
IntActiQ15011. 10 interactors.
MINTiMINT-2867283.
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

Sequence similaritiesi

Contains 1 ubiquitin-like domain.PROSITE-ProRule annotation

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

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

Family and domain databases

InterProiIPR029071. Ubiquitin-rel_dom.
IPR000626. Ubiquitin_dom.
[Graphical view]
PfamiPF00240. ubiquitin. 1 hit.
[Graphical view]
SMARTiSM00213. UBQ. 1 hit.
[Graphical view]
SUPFAMiSSF54236. SSF54236. 1 hit.
PROSITEiPS50053. UBIQUITIN_2. 1 hit.
[Graphical view]

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 rs2217332dbSNPEnsembl.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

Cross-referencesi

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.

3D structure databases

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

Protein-protein interaction databases

BioGridi115060. 29 interactors.
DIPiDIP-46662N.
IntActiQ15011. 10 interactors.
MINTiMINT-2867283.
STRINGi9606.ENSP00000409555.

PTM databases

iPTMnetiQ15011.
PhosphoSitePlusiQ15011.

Polymorphism and mutation databases

DMDMi3123034.

Proteomic databases

EPDiQ15011.
MaxQBiQ15011.
PaxDbiQ15011.
PeptideAtlasiQ15011.
PRIDEiQ15011.

Protocols and materials databases

DNASUi9709.
Structural Biology KnowledgebaseSearch...

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]

Organism-specific databases

CTDi9709.
DisGeNETi9709.
GeneCardsiHERPUD1.
HGNCiHGNC:13744. HERPUD1.
HPAiCAB037041.
CAB037104.
HPA040754.
HPA041219.
MIMi608070. gene.
neXtProtiNX_Q15011.
OpenTargetsiENSG00000051108.
PharmGKBiPA29252.
HUGEiSearch...
GenAtlasiSearch...

Phylogenomic databases

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

Enzyme and pathway databases

BioCyciZFISH:ENSG00000051108-MONOMER.
ReactomeiR-HSA-380994. ATF4 activates genes.
SIGNORiQ15011.

Miscellaneous databases

ChiTaRSiHERPUD1. human.
EvolutionaryTraceiQ15011.
GeneWikiiHERPUD1.
GenomeRNAii9709.
PROiQ15011.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000051108.
CleanExiHS_HERPUD1.
ExpressionAtlasiQ15011. baseline and differential.
GenevisibleiQ15011. HS.

Family and domain databases

InterProiIPR029071. Ubiquitin-rel_dom.
IPR000626. Ubiquitin_dom.
[Graphical view]
PfamiPF00240. ubiquitin. 1 hit.
[Graphical view]
SMARTiSM00213. UBQ. 1 hit.
[Graphical view]
SUPFAMiSSF54236. SSF54236. 1 hit.
PROSITEiPS50053. UBIQUITIN_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiHERP1_HUMAN
AccessioniPrimary (citable) accession number: Q15011
Secondary accession number(s): E9PGD1
, O60644, Q6IAN8, Q96D92
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: November 1, 1996
Last modified: November 2, 2016
This is version 158 of the entry and version 1 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

Miscellaneous

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

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 16
    Human chromosome 16: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. SIMILARITY comments
    Index of protein domains and families

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 one 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.