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Gene Review

Uchl3  -  ubiquitin carboxyl-terminal esterase L3...

Mus musculus

Synonyms: UCH-L3, Ubiquitin carboxyl-terminal hydrolase isozyme L3, Ubiquitin thioesterase L3
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Disease relevance of Uchl3


High impact information on Uchl3

  • We identify Ub C-terminal hydrolase L1 (UCH-L1) and DEN1/NEDP1/SENP8, in addition to UCH-L3, as proteases with specificity for Nedd8 [2].
  • The deletion includes Uchl3, Lmo7 and an additional 500 kb downstream of the 3' end of Lmo7 where no genes are thought to reside [3].
  • We suggest that the increase in severity may reflect an interaction between Uchl3 and Lmo7 in the ubiquitin-mediated protein degradation pathway [3].
  • We predicted that the region contains a single cluster of four genes that encode a TBC domain-containing protein (KIAA0603), a novel protein AK000009, the ubiquitin C-terminal hydrolase L3 (UCHL3) and an F-box/PDZ/LIM domain protein LMO7 [3].
  • In contrast to the UCH L3 isozyme that is universally expressed in all tissues, UCH L1 is expressed exclusively in neurons and testis/ovary [4].

Biological context of Uchl3


Anatomical context of Uchl3


Other interactions of Uchl3


Analytical, diagnostic and therapeutic context of Uchl3

  • The caput epididymides of gad mice were resistant to apoptotic stress induced by efferent duct ligation, whereas Uchl3 knockout mice showed a marked increase in apoptotic cells following ligation [7].


  1. Photoreceptor cell apoptosis in the retinal degeneration of Uchl3-deficient mice. Sano, Y., Furuta, A., Setsuie, R., Kikuchi, H., Wang, Y.L., Sakurai, M., Kwon, J., Noda, M., Wada, K. Am. J. Pathol. (2006) [Pubmed]
  2. Specific and covalent targeting of conjugating and deconjugating enzymes of ubiquitin-like proteins. Hemelaar, J., Borodovsky, A., Kessler, B.M., Reverter, D., Cook, J., Kolli, N., Gan-Erdene, T., Wilkinson, K.D., Gill, G., Lima, C.D., Ploegh, H.L., Ovaa, H. Mol. Cell. Biol. (2004) [Pubmed]
  3. An engineered 800 kilobase deletion of Uchl3 and Lmo7 on mouse chromosome 14 causes defects in viability, postnatal growth and degeneration of muscle and retina. Semenova, E., Wang, X., Jablonski, M.M., Levorse, J., Tilghman, S.M. Hum. Mol. Genet. (2003) [Pubmed]
  4. Ubiquitin carboxy-terminal hydrolase L1 binds to and stabilizes monoubiquitin in neuron. Osaka, H., Wang, Y.L., Takada, K., Takizawa, S., Setsuie, R., Li, H., Sato, Y., Nishikawa, K., Sun, Y.J., Sakurai, M., Harada, T., Hara, Y., Kimura, I., Chiba, S., Namikawa, K., Kiyama, H., Noda, M., Aoki, S., Wada, K. Hum. Mol. Genet. (2003) [Pubmed]
  5. Ubiquitin C-terminal hydrolase L3 (Uchl3) is involved in working memory. Wood, M.A., Kaplan, M.P., Brensinger, C.M., Guo, W., Abel, T. Hippocampus. (2005) [Pubmed]
  6. Two closely related ubiquitin C-terminal hydrolase isozymes function as reciprocal modulators of germ cell apoptosis in cryptorchid testis. Kwon, J., Wang, Y.L., Setsuie, R., Sekiguchi, S., Sato, Y., Sakurai, M., Noda, M., Aoki, S., Yoshikawa, Y., Wada, K. Am. J. Pathol. (2004) [Pubmed]
  7. The region-specific functions of two ubiquitin C-terminal hydrolase isozymes along the epididymis. Kwon, J., Sekiguchi, S., Wang, Y.L., Setsuie, R., Yoshikawa, Y., Wada, K. Exp. Anim. (2006) [Pubmed]
  8. Localization of ubiquitin C-terminal hydrolase l1 in mouse ova and its function in the plasma membrane to block polyspermy. Sekiguchi, S., Kwon, J., Yoshida, E., Hamasaki, H., Ichinose, S., Hideshima, M., Kuraoka, M., Takahashi, A., Ishii, Y., Kyuwa, S., Wada, K., Yoshikawa, Y. Am. J. Pathol. (2006) [Pubmed]
  9. Developmental regulation of ubiquitin C-terminal hydrolase isozyme expression during spermatogenesis in mice. Kwon, J., Wang, Y.L., Setsuie, R., Sekiguchi, S., Sakurai, M., Sato, Y., Lee, W.W., Ishii, Y., Kyuwa, S., Noda, M., Wada, K., Yoshikawa, Y. Biol. Reprod. (2004) [Pubmed]
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