UBXN1

Functionⁱ

Ubiquitin-binding protein that plays a role in the modulation of innate immune response. Blocks both the RIG-I-like receptors (RLR) and NF-kappa-B pathways. Following viral infection, UBXN1 is induced and recruited to the RLR component MAVS. In turn, interferes with MAVS oligomerization, and disrupts the MAVS/TRAF3/TRAF6 signalosome. This function probably serves as a brake to prevent excessive RLR signaling (PubMed:23545497). Interferes with the TNFalpha-triggered NF-kappa-B pathway by interacting with cellular inhibitors of apoptosis proteins (cIAPs) and thereby inhibiting their recruitment to TNFR1 (PubMed:25681446). Prevents also the activation of NF-kappa-B by associating with CUL1 and thus inhibiting NF-kappa-B inhibitor alpha/NFKBIA degradation that remains bound to NF-kappa-B (PubMed:28152074). Interacts with the BRCA1-BARD1 heterodimer and regulates its activity. Specifically binds 'Lys-6'-linked polyubiquitin chains. Interaction with autoubiquitinated BRCA1 leads to the inhibition of the E3 ubiquitin-protein ligase activity of the BRCA1-BARD1 heterodimer (PubMed:20351172). Component of a complex required to couple deglycosylation and proteasome-mediated degradation of misfolded proteins in the endoplasmic reticulum that are retrotranslocated in the cytosol.4 Publications

GO - Molecular functionⁱ

ATPase binding
Source: UniProtKB
Inferred from physical interactionⁱ
- 15362974
K48-linked polyubiquitin modification-dependent protein binding
Source: ParkinsonsUK-UCL
Inferred from direct assayⁱ
- 21135095
K6-linked polyubiquitin modification-dependent protein binding
Source: UniProtKB
Inferred from direct assayⁱ
- 20351172
polyubiquitin modification-dependent protein binding
Source: UniProtKB
Inferred from direct assayⁱ
- 15362974
ubiquitin binding
Source: BHF-UCL
Inferred from direct assayⁱ
- 18775313
ubiquitin protein ligase binding
Source: BHF-UCL
Inferred from direct assayⁱ
- 18775313

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

negative regulation of ERAD pathway
Source: ParkinsonsUK-UCL
Inferred from mutant phenotypeⁱ
- 21135095
negative regulation of proteasomal ubiquitin-dependent protein catabolic process
Source: UniProtKB
Inferred from direct assayⁱ
- 20351172
negative regulation of protein K48-linked deubiquitination
Source: ParkinsonsUK-UCL
Inferred from direct assayⁱ
- 21135095
negative regulation of protein ubiquitination
Source: UniProtKB
Inferred from direct assayⁱ
- 20351172
negative regulation of ubiquitin-specific protease activity
Source: ParkinsonsUK-UCL
Inferred from direct assayⁱ
- 21135095
proteasome-mediated ubiquitin-dependent protein catabolic process
Source: UniProtKB
Non-traceable author statementⁱ
- 15362974
protein folding Source: Reactome
viral process Source: UniProtKB-KW

View the complete GO annotation on QuickGO ...

Keywordsⁱ

Biological process	Host-virus interaction

Biological process

Host-virus interaction

Enzyme and pathway databases

Reactomeⁱ	R-HSA-532668 N-glycan trimming in the ER and Calnexin/Calreticulin cycle

Reactomeⁱ

R-HSA-532668 N-glycan trimming in the ER and Calnexin/Calreticulin cycle

Names & Taxonomyⁱ

Protein namesⁱ	Recommended name: UBX domain-containing protein 1 Alternative name(s): SAPK substrate protein 1 UBA/UBX 33.3 kDa protein
Gene namesⁱ	Name:UBXN1 Synonyms:SAKS1
Organismⁱ	Homo sapiens (Human)
Taxonomic identifierⁱ	9606 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo
Proteomesⁱ	UP000005640 Componentⁱ: Chromosome 11

Organism-specific databases

EuPathDBⁱ	HostDB:ENSG00000162191.13
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:18402 UBXN1
MIMⁱ	616378 gene
neXtProtⁱ	NX_Q04323

Keywords - Cellular componentⁱ

Cytoplasm

Pathology & Biotechⁱ

Mutagenesis

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Mutagenesisⁱ	13	M → T: Abolishes binding to 'Lys-6'-linked polyubiquitin chains and ability to inhibit E3 ubiquitin-protein ligase activity of the BRCA1-BARD1 heterodimer. 1 Publication		1
Mutagenesisⁱ	219	R → A: Does not affect binding to 'Lys-6'-linked polyubiquitin chains and ability to inhibit E3 ubiquitin-protein ligase activity of the BRCA1-BARD1 heterodimer. 1 Publication		1

Organism-specific databases

DisGeNET More...DisGeNETⁱ	51035
Open Targets More...OpenTargetsⁱ	ENSG00000162191
PharmGKBⁱ	PA162408336

Polymorphism and mutation databases

BioMutaⁱ	UBXN1
DMDMⁱ	30923268

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Initiator methionineⁱ		RemovedCombined sources
ChainⁱPRO_0000211023	2 – 297	UBX domain-containing protein 1Add BLAST		296

Amino acid modifications

Feature key	Position(s)	DescriptionActions	Length
Modified residueⁱ	2	N-acetylalanineCombined sources	1
Modified residueⁱ	199	PhosphoserineCombined sources	1
Modified residueⁱ	200	Phosphoserine; by MAPK121 Publication	1
Modified residueⁱ	207	PhosphothreonineCombined sources	1
Modified residueⁱ	229	PhosphothreonineCombined sources	1
Modified residueⁱ	270	PhosphoserineCombined sources	1

Keywords - PTMⁱ

Acetylation, Phosphoprotein

Proteomic databases

EPDⁱ	Q04323
MaxQB - The MaxQuant DataBase More...MaxQBⁱ	Q04323
PaxDbⁱ	Q04323
PeptideAtlas More...PeptideAtlasⁱ	Q04323
PRIDEⁱ	Q04323
ProteomicsDBⁱ	58240 58241 [Q04323-2]

PTM databases

iPTMnetⁱ	Q04323
PhosphoSitePlusⁱ	Q04323

Expressionⁱ

Gene expression databases

Bgeeⁱ	ENSG00000162191 Expressed in 231 organ(s), highest expression level in testis
CleanExⁱ	HS_UBXN1
ExpressionAtlasⁱ	Q04323 baseline and differential
Genevisibleⁱ	Q04323 HS

Organism-specific databases

Human Protein Atlas More...HPAⁱ	HPA012669

HPAⁱ

HPA012669

Interactionⁱ

Subunit structureⁱ

Interacts with MAVS; this interaction prevents MAVS oligomerization and thus disrupts the RLR signaling pathway (PubMed:23545497). Interacts with CUL1; this interaction inhibits CUL1-mediated degradation of NF-kappa-B inhibitors (PubMed:28152074). Interacts with BIRC2/c-IAP1; this interaction prevents TNFalpha-stimulated RIP1 ubiquitination and subsequent NF-kappa-B activation (PubMed:25681446). Component of a complex required to couple retrotranslocation, ubiquitination and deglycosylation composed of NGLY1, SAKS1, AMFR, VCP and RAD23B. Interacts with HOMER2 (By similarity). Interacts directly with VCP. Interacts with BRCA1 and BARD1; interaction takes place when BRCA1 is not autoubiquitinated but is strongly enhanced in the presence of autoubiquitinated BRCA1.By similarity6 Publications

(Microbial infection) Interacts (via C-terminal UBX domain) with nipah virus protein V.1 Publication

Binary interactionsⁱ

With	Entry	#Exp.	IntAct
	Q8WU02	3	EBI-1058647,EBI-747182
HEL-S-70	V9HW80	3	EBI-1058647,EBI-10175326
PLAA	Q9Y263	5	EBI-1058647,EBI-1994037
TRIM39	Q9HCM9	3	EBI-1058647,EBI-739510
UBE2I	Q7KZS0	3	EBI-1058647,EBI-10180829
UBQLN1	Q9UMX0	3	EBI-1058647,EBI-741480
UBQLN1	Q9UMX0-2	3	EBI-1058647,EBI-10173939

GO - Molecular functionⁱ

ATPase binding
Source: UniProtKB
Inferred from physical interactionⁱ
- 15362974
K48-linked polyubiquitin modification-dependent protein binding
Source: ParkinsonsUK-UCL
Inferred from direct assayⁱ
- 21135095
K6-linked polyubiquitin modification-dependent protein binding
Source: UniProtKB
Inferred from direct assayⁱ
- 20351172
polyubiquitin modification-dependent protein binding
Source: UniProtKB
Inferred from direct assayⁱ
- 15362974
ubiquitin binding
Source: BHF-UCL
Inferred from direct assayⁱ
- 18775313
ubiquitin protein ligase binding
Source: BHF-UCL
Inferred from direct assayⁱ
- 18775313

View the complete GO annotation on QuickGO ...

Protein-protein interaction databases

BioGridⁱ	119239, 147 interactors
Database of interacting proteins More...DIPⁱ	DIP-29467N
IntActⁱ	Q04323, 42 interactors
STRINGⁱ	9606.ENSP00000294119

Structureⁱ

3D structure databases

ProteinModelPortalⁱ	Q04323
SMRⁱ	Q04323
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Family & Domainsⁱ

Domains and Repeats

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Domainⁱ	2 – 42	UBAPROSITE-ProRule annotationAdd BLAST		41
Domainⁱ	211 – 293	UBXPROSITE-ProRule annotationAdd BLAST		83

Region

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Regionⁱ	43 – 297	Interaction with BRCA11 PublicationAdd BLAST		255

Coiled coil

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Coiled coilⁱ	87 – 172	Sequence analysisAdd BLAST		86

Domainⁱ

The UBA domain specifically recognizes and binds 'Lys-6'-linked polyubiquitin chains.1 Publication

Keywords - Domainⁱ

Coiled coil

Phylogenomic databases

eggNOGⁱ	KOG2689 Eukaryota ENOG4111HH6 LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00530000063500
HOGENOMⁱ	HOG000188321
HOVERGENⁱ	HBG082310
InParanoidⁱ	Q04323
OMAⁱ	PSAVITM
PhylomeDBⁱ	Q04323
TreeFamⁱ	TF313944

Family and domain databases

InterProⁱ	View protein in InterPro IPR015940 UBA IPR009060 UBA-like_sf IPR029071 Ubiquitin-like_domsf IPR001012 UBX_dom
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF00627 UBA, 1 hit PF00789 UBX, 1 hit
SMARTⁱ	View protein in SMART SM00165 UBA, 1 hit SM00166 UBX, 1 hit
SUPFAMⁱ	SSF46934 SSF46934, 1 hit SSF54236 SSF54236, 1 hit
PROSITEⁱ	View protein in PROSITE PS50030 UBA, 1 hit PS50033 UBX, 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. Align Add to basket

This entry has 2 described isoforms and 4 potential isoforms that are computationally mapped.ⁱShow all

Isoform 1 (identifier: Q04323-1) [UniParc]FASTA Add 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
MAELTALESL IEMGFPRGRA EKALALTGNQ GIEAAMDWLM EHEDDPDVDE 
        60         70         80         90        100
PLETPLGHIL GREPTSSEQG GLEGSGSAAG EGKPALSEEE RQEQTKRMLE 
       110        120        130        140        150
LVAQKQRERE EREEREALER ERQRRRQGQE LSAARQRLQE DEMRRAAEER 
       160        170        180        190        200
RREKAEELAA RQRVREKIER DKAERAKKYG GSVGSQPPPV APEPGPVPSS 
       210        220        230        240        250
PSQEPPTKRE YDQCRIQVRL PDGTSLTQTF RAREQLAAVR LYVELHRGEE 
       260        270        280        290 
LGGGQDPVQL LSGFPRRAFS EADMERPLQE LGLVPSAVLI VAKKCPS

Length:297

Mass (Da):33,325

Last modified:May 16, 2003 - v2

Checksum:ⁱE4E0C8BBAC93F2BB

GO

Isoform 2 (identifier: Q04323-2) [UniParc]FASTA Add to basket

The sequence of this isoform differs from the canonical sequence as follows:
283-297: LVPSAVLIVAKKCPS → MAARLETRTRNWGSREACLGKGGMQREGAL

« Hide

        10         20         30         40         50
MAELTALESL IEMGFPRGRA EKALALTGNQ GIEAAMDWLM EHEDDPDVDE 
        60         70         80         90        100
PLETPLGHIL GREPTSSEQG GLEGSGSAAG EGKPALSEEE RQEQTKRMLE 
       110        120        130        140        150
LVAQKQRERE EREEREALER ERQRRRQGQE LSAARQRLQE DEMRRAAEER 
       160        170        180        190        200
RREKAEELAA RQRVREKIER DKAERAKKYG GSVGSQPPPV APEPGPVPSS 
       210        220        230        240        250
PSQEPPTKRE YDQCRIQVRL PDGTSLTQTF RAREQLAAVR LYVELHRGEE 
       260        270        280        290        300
LGGGQDPVQL LSGFPRRAFS EADMERPLQE LGMAARLETR TRNWGSREAC 
       310 
LGKGGMQREG AL

Note: No experimental confirmation available.

Show »

Length:312

Mass (Da):35,107

Checksum:ⁱ75C94CB67C03EB78

GO

Computationally mapped potential isoform sequencesⁱ

There are 4 potential isoforms mapped to this entry.BLAST Align Show all Add to basket

Entry	Entry name	Protein names	Gene names	Length	Annotation
A0A087WTZ5	A0A087WTZ5_HUMAN	UBX domain-containing protein 1 UBX domain-containing protein 1	UBXN1	238	Annotation score:
E9PRQ7	E9PRQ7_HUMAN	UBX domain-containing protein 1 UBX domain-containing protein 1	UBXN1	293	Annotation score:
E9PJ81	E9PJ81_HUMAN	UBX domain-containing protein 1 UBX domain-containing protein 1	UBXN1	290	Annotation score:
E9PS08	E9PS08_HUMAN	UBX domain-containing protein 1 UBX domain-containing protein 1	UBXN1	100	Annotation score:

Experimental Info

Feature key	Position(s)	DescriptionActions	Length
Sequence conflictⁱ	76	G → A in AAA36396 (Ref. 1) Curated	1
Sequence conflictⁱ	148	E → AE in AAA36396 (Ref. 1) Curated	1
Sequence conflictⁱ	154	K → N in AAA36396 (Ref. 1) Curated	1

Natural variant

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Natural variantⁱVAR_057370	56	L → F. Corresponds to variant dbSNP:rs11543359Ensembl.		1

Alternative sequence

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Alternative sequenceⁱVSP_020367	283 – 297	LVPSA…KKCPS → MAARLETRTRNWGSREACLG KGGMQREGAL in isoform 2. 1 PublicationAdd BLAST		15

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	M68864 mRNA Translation: AAA36396.1 BC000902 mRNA Translation: AAH00902.1 BC001372 mRNA Translation: AAH01372.1 BC040129 mRNA Translation: AAH40129.1
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS66105.1 [Q04323-1] CCDS8029.1 [Q04323-2]
PIRⁱ	S27965
NCBI Reference Sequences More...RefSeqⁱ	NP_001273006.1, NM_001286077.1 [Q04323-1] NP_001273007.1, NM_001286078.1 NP_056937.2, NM_015853.4 [Q04323-2] XP_005274090.1, XM_005274033.4 [Q04323-2] XP_016873363.1, XM_017017874.1 [Q04323-1]
UniGeneⁱ	Hs.351296 Hs.731509

Genome annotation databases

Ensemblⁱ	ENST00000294119; ENSP00000294119; ENSG00000162191 [Q04323-2] ENST00000301935; ENSP00000303991; ENSG00000162191 [Q04323-1]
GeneIDⁱ	51035
KEGGⁱ	hsa:51035
UCSC genome browser More...UCSCⁱ	uc001nuj.5 human [Q04323-1]

Keywords - Coding sequence diversityⁱ

Alternative splicing, Polymorphism

Similar proteinsⁱ

100% Identity
90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length	Source
Q04323	Uncharacterized protein		HUMAN		297	UniRef100_Q04323
UBX domain-containing protein 1 (Fragment)		HUMAN		290
cDNA FLJ53007, highly similar to UBA/UBX 33.3 kDa protein		HUMAN		200

Protein	Similar proteins		Species	Score	Length	Source
Q04323	Uncharacterized protein		HUMAN		297	UniRef90_Q04323
UBX domain-containing protein 1 isoform X1		ERIEU		298
UBX domain protein 1		RHIBE		297
UBX domain protein 1		MACMU		297
UBXN1 isoform 1		PONAB		297
+71
Q04323-2	UBX domain protein 1		PANTR		310	UniRef90_H2Q3W4
UBX domain protein 1		PANPA		310
Uncharacterized protein		HUMAN		312
UBX domain protein 1		GORGO		310
UBXN1 isoform 3		PONAB		310
+19

Protein	Similar proteins		Species	Score	Length	Source
Q04323	Uncharacterized protein		HUMAN		297	UniRef50_Q04323
UBX domain-containing protein 1 isoform X1		ERIEU		298
UBX domain protein 1		OTOGA		297
UBX domain protein 1		GORGO		297
UBX domain protein 1		LOXAF		297
+80
Q04323-2	UBX domain protein 1		PANTR		310	UniRef50_H2Q3W4
UBX domain protein 1		PANPA		310
Uncharacterized protein		HUMAN		312
UBX domain protein 1		RHIRO		310
UBX domain protein 1		Cebus capucinus imitator		310
+20

Entry informationⁱ

Entry nameⁱ	UBXN1_HUMAN
Accessionⁱ	Q04323Primary (citable) accession number: Q04323 Secondary accession number(s): Q9BV93, Q9BVV5
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	October 1, 1996
	Last sequence update:	May 16, 2003
	Last modified:	September 12, 2018
	This is version 153 of the entry and version 2 of the sequence. See complete history.
Entry statusⁱ	Reviewed (UniProtKB/Swiss-Prot)
Annotation program	Chordata Protein Annotation Program
Disclaimer	Any 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

Complete proteome, Direct protein sequencing, Reference proteome

UniProtKB

UniParc

Help

UniRef

Proteomes

Annotation systems

Supporting data

UniProtKB - Q04323 (UBXN1_HUMAN)

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UBX domain-containing protein 1

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<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

Enzyme and pathway databases

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

Organism-specific databases

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Other locations

Cytosol

Endoplasmic reticulum

Nucleus

Other locations

Keywords - Cellular componenti

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

Mutagenesis

Organism-specific databases

Polymorphism and mutation databases

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Amino acid modifications

Keywords - PTMi

Proteomic databases

PTM databases

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Organism-specific databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

GO - Molecular functioni

Protein-protein interaction databases

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Domains and Repeats

Region

Coiled coil

Keywords - Domaini

Phylogenomic databases

Family and domain databases

Computationally mapped potential isoform sequencesi

Experimental Info

Natural variant

Alternative sequence

Sequence databases

Genome annotation databases

Keywords - Coding sequence diversityi

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

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Functionⁱ

GO - Molecular functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Keywords - Cellular componentⁱ

Pathology & Biotechⁱ

PTM / Processingⁱ

Keywords - PTMⁱ

Expressionⁱ

Interactionⁱ

GO - Molecular functionⁱ

Structureⁱ

Family & Domainsⁱ

Keywords - Domainⁱ

Computationally mapped potential isoform sequencesⁱ

Keywords - Coding sequence diversityⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ