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Gene Review

UBQLN4  -  ubiquilin 4

Homo sapiens

Synonyms: A1U, A1Up, Ataxin-1 interacting ubiquitin-like protein, Ataxin-1 ubiquitin-like-interacting protein A1U, C1orf6, ...
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High impact information on UBQLN4

  • These results suggest that A1Up may link ataxin-1 with the chaperone and ubiquitin-proteasome pathways [1].
  • A1Up localized to the nucleus and cytoplasm of transfected COS-1 cells [1].
  • We also demonstrate that the cDNA sequence shown as part of the LIS2 gene and marking its chromosome 2 specificity belongs to the 3' untranslated region of a different gene (C1orf6) that we mapped to 1q21 by FISH analysis [2].

Anatomical context of UBQLN4

  • Expression analyses demonstrated that A1U mRNA is widely expressed as an approximately 4.0 kb transcript and is present in Purkinje cells, the primary site of SCA1 cerebellar pathology [1].

Analytical, diagnostic and therapeutic context of UBQLN4

  • Molecular cloning of a novel ubiquitin-like protein, UBIN, that binds to ER targeting signal sequences [3].


  1. Identification and characterization of an ataxin-1-interacting protein: A1Up, a ubiquitin-like nuclear protein. Davidson, J.D., Riley, B., Burright, E.N., Duvick, L.A., Zoghbi, H.Y., Orr, H.T. Hum. Mol. Genet. (2000) [Pubmed]
  2. Identification of two paralogous regions mapping to the short and long arms of human chromosome 2 comprising LIS1 pseudogenes. Fogli, A., Giglio, S., Arrigo, G., Lo Nigro, C., Zollo, M., Viggiano, L., Rocchi, M., Archidiacono, N., Zuffardi, O., Carrozzo, R. Cytogenet. Cell Genet. (1999) [Pubmed]
  3. Molecular cloning of a novel ubiquitin-like protein, UBIN, that binds to ER targeting signal sequences. Matsuda, M., Koide, T., Yorihuzi, T., Hosokawa, N., Nagata, K. Biochem. Biophys. Res. Commun. (2001) [Pubmed]
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