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Protein UBQLN1 PDB 2JY5.png
Available structures
PDB Ortholog search: PDBe RCSB
Aliases UBQLN1, DA41, DSK2, PLIC-1, UBQN, XDRP1, ubiquilin 1
External IDs MGI: 1860276 HomoloGene: 137258 GeneCards: UBQLN1
Species Human Mouse
RefSeq (mRNA)



RefSeq (protein)



Location (UCSC) Chr 9: 83.66 – 83.71 Mb Chr 13: 58.18 – 58.22 Mb
PubMed search [1] [2]
View/Edit Human View/Edit Mouse

Ubiquilin-1 is a protein that in humans is encoded by the UBQLN1 gene.[3][4][5]

Ubiquilins contain a 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.

Possible Role In Preventing Alzheimers Disease[edit]

Ubiquilin has also been shown to modulate accumulation of presenilin proteins, and is found in lesions associated with Alzheimer's and Parkinson's disease. Two transcript variants encoding different isoforms have been found for this gene.[5]

Higher levels of ubiquilin-1 in the brain decreased malformation of the APP molecule which plays a key role in triggering Alzheimers disease.[6] Conversely, lower levels of ubiquilin-1 in the brain were associated with increased malformation of APP.[6]

Similarity to Other Proteins[edit]

Human UBQLN1 shares a high degree of similarity with related ubiquilins including UBQLN2 and UBQLN4.[7]


UBQLN1 has been shown to interact with


  1. ^ "Human PubMed Reference:". 
  2. ^ "Mouse PubMed Reference:". 
  3. ^ Ozaki T, Hishiki T, Toyama Y, Yuasa S, Nakagawara A, Sakiyama S (Oct 1997). "Identification of a new cellular protein that can interact specifically with DAN". DNA Cell Biol. 16 (8): 985–91. doi:10.1089/dna.1997.16.985. PMID 9303440. 
  4. ^ Hanaoka E, Ozaki T, Ohira M, Nakamura Y, Suzuki M, Takahashi E, Moriya H, Nakagawara A, Sakiyama S (Jul 2000). "Molecular cloning and expression analysis of the human DA41 gene and its mapping to chromosome 9q21.2-q21.3". J Hum Genet. 45 (3): 188–91. doi:10.1007/s100380050209. PMID 10807547. 
  5. ^ a b "Entrez Gene: UBQLN1 ubiquilin 1". 
  6. ^ a b Stieren ES, El Ayadi A, Xiao Y, Siller E, Landsverk ML, Oberhauser AF, Barral JM, Boehning D (August 2011). "Ubiquilin-1 Is a Molecular Chaperone for the Amyloid Precursor Protein". J Biol Chem. 286 (41): 35689–98. doi:10.1074/jbc.M111.243147. PMC 3195644Freely accessible. PMID 21852239. Lay summaryScience Daily. 
  7. ^ Marín I (March 2014). "The ubiquilin gene family: evolutionary patterns and functional insights". BMC Evol Biol. 14: 63. doi:10.1186/1471-2148-14-63. PMID 24674348. 
  8. ^ Kim TY, Kim E, Yoon SK, Yoon JB (May 2008). "Herp enhances ER-associated protein degradation by recruiting ubiquilins". Biochem. Biophys. Res. Commun. 369 (2): 741–6. doi:10.1016/j.bbrc.2008.02.086. PMID 18307982. 
  9. ^ Wu S, Mikhailov A, Kallo-Hosein H, Hara K, Yonezawa K, Avruch J (January 2002). "Characterization of ubiquilin 1, an mTOR-interacting protein". Biochim. Biophys. Acta. 1542 (1–3): 41–56. doi:10.1016/S0167-4889(01)00164-1. PMID 11853878. 
  10. ^ Ko HS, Uehara T, Nomura Y (September 2002). "Role of ubiquilin associated with protein-disulfide isomerase in the endoplasmic reticulum in stress-induced apoptotic cell death". J. Biol. Chem. 277 (38): 35386–92. doi:10.1074/jbc.M203412200. PMID 12095988. 
  11. ^ a b Mah AL, Perry G, Smith MA, Monteiro MJ (November 2000). "Identification of Ubiquilin, a Novel Presenilin Interactor That Increases Presenilin Protein Accumulation". J. Cell Biol. 151 (4): 847–62. doi:10.1083/jcb.151.4.847. PMC 2169435Freely accessible. PMID 11076969. 
  12. ^ Kleijnen MF, Shih AH, Zhou P, Kumar S, Soccio RE, Kedersha NL, Gill G, Howley PM (August 2000). "The hPLIC proteins may provide a link between the ubiquitination machinery and the proteasome". Mol. Cell. 6 (2): 409–19. doi:10.1016/S1097-2765(00)00040-X. PMID 10983987. 
  13. ^ Rolland, T. et al. "A proteome-scale map of the human interactome network.", Cell, USA, 2014 November 20. Retrieved on 28 April 2015.

Further reading[edit]