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Cited by 251Nerve injury and regenerationHereditary Neurological DisordersAutophagy in Disease and Therapy

A Ufd2/D4Cole1e chimeric protein and overexpression of Rbp7 in the slow Wallerian degeneration ( Wld S ) mouse

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Conforti, Laura, Tarlton, Andrea, Mack, Till G. A., Mi, Weiqian, Buckmaster, E. Anne, Wagner, Diana, Perry, V. Hugh, Coleman, Michael P.·2000·Proceedings of the National Academy of Sciences
DOI 10.1073/pnas.97.21.11377PMC PMC17208
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Abstract

Exons of three genes were identified within the 85-kilobase tandem triplication unit of the slow Wallerian degeneration mutant mouse, C57BL/ Wld S . Ubiquitin fusion degradation protein 2 ( Ufd2 ) and a previously undescribed gene, D4Cole1e , span the proximal and distal boundaries of the repeat unit, respectively. They have the same chromosomal orientation and form a chimeric gene when brought together at the boundaries between adjacent repeat units in Wld S . The chimeric mRNA is abundantly expressed in the nervous system and encodes an in-frame fusion protein consisting of the N-terminal 70 amino acids of Ufd2 , the C-terminal 302 amino acids of D4Cole1e , and an aspartic acid formed at the junction. Antisera raised against synthetic peptides detect the expected 43-kDa protein specifically in Wld S brain. This expression pattern, together with the previously established role of ubiquitination in axon degeneration, makes the chimeric gene a promising candidate for Wld . The third gene altered by the triplication, Rbp7 , is a novel member of the cellular retinoid-binding protein family and is highly expressed in white adipose tissue and mammary gland. The whole gene lies within the repeat unit leading to overexpression of the normal transcript in Wld S mice. However, it is undetectable on Northern blots of Wld S brain and seems unlikely to be the Wld gene. These data reveal both a candidate gene for Wld and the potential of the Wld S mutant for studies of ubiquitin and retinoid metabolism.

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  • The protective effects in WldS mice are attributed to a chimeric gene Ube4b/NMNAT1, which encodes an in-frame fusion protein of the amino-terminal of UBE4B and the complete coding region of NMNAT1.

    “The protective effects occurring in these mice are attributed to a chimeric gene Ube4b/NMNAT1, which encodes an in-frame fusion protein of the amino-terminal of ubiquitination factor E4B (UBE4B) and the complete coding region of NMNAT1 (83,84).”

    From CREB-activity and nmnat2 transcription are down-regulated prior to neurodegeneration, while NMNAT2 over-expression is neuroprotective, in a mouse model of human tauopathy (2012) · cites this paper as [83]

    The Results identify a WldS-specific in-frame fusion of the N-terminal 70 amino acids of Ufd2 and the C-terminal 302 amino acids of D4Cole1e. The Abstract and Discussion call it a promising candidate for slowing Wallerian degeneration, not an established cause of protection. Our copy does not identify D4Cole1e as NMNAT1 or say that the fusion contains NMNAT1’s complete coding region. (LLM, conf=0.88)

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Cited by

Sorted by citations

Authors
Conforti, L., Tarlton, A., Mack, T. G. A., Mi, W., Buckmaster, E. A., Wagner, D., Perry, V. H., & Coleman, M. P.
Affiliations
University of Oxford (GB)
ORCID
Venue
Proceedings of the National Academy of Sciences
Publisher
National Academy of Sciences
Published
2000-10-10
Volume
97
Issue
21
Pages
11377-11382
ISSN
  • 0027-8424 (ISSN-L)
  • 1091-6490
DOI
10.1073/pnas.97.21.11377
PMC
PMC17208
OpenAlex
W2149931901
Cited by
251
References
0
Citations by year
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FWCI
5.03
Type
journal article
Language
en
Open access
Locations
  • Proceedings of the National Academy of Sciences (publishedVersion)
  • Europe PMC (PubMed Central) (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
  • ePrints Soton (University of Southampton) (submittedVersion)
Topics
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Nerve injury and regeneration
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
  • Health Sciences › Medicine › Epidemiology › Autophagy in Disease and Therapy
Keywords
fusion genes, axonal degeneration, ubiquitination, vitamin A metabolism, candidate genes
MeSH terms
Amino Acid Sequence, Animals, Base Sequence, Central Nervous System, Exons, Fungal Proteins, Mice, Inbred C57BL, Molecular Sequence Data, Nicotinamide-Nucleotide Adenylyltransferase, Proteins, Recombinant Fusion Proteins, Retinol-Binding Proteins, RNA, Messenger, Wallerian Degeneration, Sequence Homology, Amino Acid, Peripheral Nervous System, DNA, Complementary, Saccharomyces cerevisiae Proteins, Ubiquitin-Conjugating Enzymes, Mice, Retinol-Binding Proteins, Cellular

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