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Gene Review

Klhl1  -  kelch-like 1

Mus musculus

Synonyms: Kelch-like protein 1
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Disease relevance of Klhl1

  • Haploinsufficiency of kelch-like protein homolog 10 causes infertility in male mice [1].
  • Effects of deletions of kelch-like genes on cowpox virus biological properties [2].

High impact information on Klhl1

  • In pre-chondrogenic cell line ATDC5 cells, furthermore, expression of Nrf2 mRNA was also confirmed together with mRNA expression of the Kelch-like ECH associating protein 1 and small Maf proteins [3].
  • Kelch-like 10 (KLHL10) is a member of the BTB (Bric-a-brac, Tramtrack, and Broad-Complex)-kelch protein superfamily essential for spermiogenesis and male fertility [4].
  • In silico analyses of rat expressed sequence tag (EST) clones corresponding to rat Klhl ortholog also indicate that its expression is also restricted to rat muscle tissues, suggesting a conserved role of klhl in vertebrates [5].
  • We found that the mouse Klhl1as RNA is not spliced and terminates in a polyadenylation site in the Klhl1 promoter region, whereas both the present and previous work show that human KLHL1AS is highly variably spliced into processed transcripts that contain up to six exons [6].
  • Mice with Klhl1 specifically deleted in only Purkinje cells had a loss of motor coordination that was almost identical to the total-tissue deletion mice [7].

Other interactions of Klhl1


  1. Haploinsufficiency of kelch-like protein homolog 10 causes infertility in male mice. Yan, W., Ma, L., Burns, K.H., Matzuk, M.M. Proc. Natl. Acad. Sci. U.S.A. (2004) [Pubmed]
  2. Effects of deletions of kelch-like genes on cowpox virus biological properties. Kochneva, G., Kolosova, I., Maksyutova, T., Ryabchikova, E., Shchelkunov, S. Arch. Virol. (2005) [Pubmed]
  3. Nuclear factor E2 p45-related factor 2 negatively regulates chondrogenesis. Hinoi, E., Takarada, T., Fujimori, S., Wang, L., Iemata, M., Uno, K., Yoneda, Y. Bone (2007) [Pubmed]
  4. Cullin3 is a KLHL10-interacting protein preferentially expressed during late spermiogenesis. Wang, S., Zheng, H., Esaki, Y., Kelly, F., Yan, W. Biol. Reprod. (2006) [Pubmed]
  5. A novel zebrafish kelchlike gene klhl and its human ortholog KLHL display conserved expression patterns in skeletal and cardiac muscles. Wu, Y.L., Gong, Z. Gene (2004) [Pubmed]
  6. The KLHL1-antisense transcript ( KLHL1AS) is evolutionarily conserved. Benzow, K.A., Koob, M.D. Mamm. Genome (2002) [Pubmed]
  7. Targeted deletion of a single Sca8 ataxia locus allele in mice causes abnormal gait, progressive loss of motor coordination, and Purkinje cell dendritic deficits. He, Y., Zu, T., Benzow, K.A., Orr, H.T., Clark, H.B., Koob, M.D. J. Neurosci. (2006) [Pubmed]
  8. Purification, crystallization and preliminary X-ray diffraction analysis of the Kelch-like motif region of mouse Keap1. Padmanabhan, B., Scharlock, M., Tong, K.I., Nakamura, Y., Kang, M.I., Kobayashi, A., Matsumoto, T., Tanaka, A., Yamamoto, M., Yokoyama, S. Acta Crystallograph. Sect. F Struct. Biol. Cryst. Commun. (2005) [Pubmed]
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