Ubiquilin-1 is a protein that in humans is encoded by the UBQLN1 gene.[5][6][7]

UBQLN1
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesUBQLN1, DA41, DSK2, PLIC-1, UBQN, XDRP1, ubiquilin 1
External IDsOMIM: 605046; MGI: 1860276; HomoloGene: 137258; GeneCards: UBQLN1; OMA:UBQLN1 - orthologs
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_013438
NM_053067

NM_026842
NM_152234

RefSeq (protein)

NP_038466
NP_444295
NP_444295.1

NP_081118
NP_689420

Location (UCSC)Chr 9: 83.66 – 83.71 MbChr 13: 58.32 – 58.36 Mb
PubMed search[3][4]
Wikidata
View/Edit HumanView/Edit Mouse

Ubiquilins contain two domains, an N-terminal ubiquitin-like domain and a C-terminal ubiquitin-associated domain. They physically associate with both proteasomes and ubiquitin ligases, and thus are thought to functionally link the ubiquitination machinery to the proteasome to effect in vivo protein degradation.

Functions

Ubiquilin-1 is associated with protein degradation and aggregation of misfolded proteins, and may be involved in neurodegenerative diseases.[8][9] Ubiquilin-1 has been reported to act as a molecular chaperone for amyloid precursor protein (APP), a protein associated with Alzheimer's disease.[10]

Ubiquilin-1 was first identified through its interactions with presenilins.[11] Two transcript variants encoding different isoforms have been found for this gene.[7]

Human UBQLN1 shares a high degree of similarity with related ubiquilins including UBQLN2 and UBQLN4.[12]

Interactions

UBQLN1 has been shown to interact with

References

Further reading