Related Content
Search Google Scholar for:
More Information
Related Jobs from ScienceCareers
|
|
Science 31 January 2003:
Vol. 299. no. 5607, pp. 710 - 712
DOI: 10.1126/science.1079694
|
|
Reports
Spongiform Degeneration in mahoganoid Mutant Mice
Lin He,1
Xin-Yun Lu,3
Aaron F. Jolly,4
Adam G. Eldridge,2
Stanley J. Watson,3
Peter K. Jackson,2
Gregory S. Barsh,1*
Teresa M. Gunn4*
mahoganoid is a mouse coat-color mutation
whose pigmentary phenotype and genetic interactions resemble those of
Attractin (Atrn). Atrn mutations also
cause spongiform neurodegeneration. Here, we show that a null mutation
for mahoganoid causes a similar age-dependent neuropathology
that includes many features of prion diseases but without accumulation
of protease-resistant prion protein. The gene mutated in
mahoganoid encodes a RING-containing protein with E3
ubiquitin ligase activity in vitro. Similarities in phenotype,
expression, and genetic interactions suggest that mahoganoid
and Atrn genes are part of a conserved pathway for regulated
protein turnover whose function is essential for neuronal viability.
1 Department of Pediatrics, Department of
Genetics, Howard Hughes Medical Institute,
2 Department of Pathology, Stanford University
School of Medicine, Stanford, CA 94305, USA.
3 Mental Health Research Institute, University of
Michigan School of Medicine, Ann Arbor, MI 48109, USA.
4 Department of Biomedical Sciences, T4 018 VRT,
Cornell University, Ithaca, NY 14853, USA.
*
To whom correspondence should be addressed. E-mail:
gbarsh{at}cmgm.stanford.edu (G.S.B.); tmg25{at}cornell.edu
(T.M.G.)
Read the Full Text
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
- Mahogunin Ring Finger-1 (MGRN1) E3 Ubiquitin Ligase Inhibits Signaling from Melanocortin Receptor by Competition with G{alpha}s.
- A. B. Perez-Oliva, C. Olivares, C. Jimenez-Cervantes, and J. C. Garcia-Borron (2009)
J. Biol. Chem.
284, 31714-31725
| Abstract »
| Full Text »
| PDF »
- Inaugural Article: A mouse forward genetics screen identifies LISTERIN as an E3 ubiquitin ligase involved in neurodegeneration.
- J. Chu, N. A. Hong, C. A. Masuda, B. V. Jenkins, K. A. Nelms, C. C. Goodnow, R. J. Glynne, H. Wu, E. Masliah, C. A. P. Joazeiro, et al. (2009)
PNAS
106, 2097-2103
| Abstract »
| Full Text »
| PDF »
- The serine protease Corin is a novel modifier of the agouti pathway.
- D. Enshell-Seijffers, C. Lindon, and B. A. Morgan (2008)
Development
135, 217-225
| Abstract »
| Full Text »
| PDF »
- Dmbx1 is essential in agouti-related protein action.
- W. Fujimoto, T. Shiuchi, T. Miki, Y. Minokoshi, Y. Takahashi, A. Takeuchi, K. Kimura, M. Saito, T. Iwanaga, and S. Seino (2007)
PNAS
104, 15514-15519
| Abstract »
| Full Text »
| PDF »
- Spongiform Neurodegeneration-associated E3 Ligase Mahogunin Ubiquitylates TSG101 and Regulates Endosomal Trafficking.
- B. Y. Kim, J. A. Olzmann, G. S. Barsh, L.-S. Chin, and L. Li (2007)
Mol. Biol. Cell
18, 1129-1142
| Abstract »
| Full Text »
| PDF »
- Emerging Roles for Ubiquitin and Protein Degradation in Neuronal Function.
- J. J. Yi and M. D. Ehlers (2007)
Pharmacol. Rev.
59, 14-39
| Abstract »
| Full Text »
| PDF »
- Heart Failure and Protein Quality Control.
- X. Wang and J. Robbins (2006)
Circ. Res.
99, 1315-1328
| Abstract »
| Full Text »
| PDF »
- The mahoganoid mutation (Mgrn1md) improves insulin sensitivity in mice with mutations in the melanocortin signaling pathway independently of effects on adiposity.
- L. K. Phan, W. K. Chung, and R. L. Leibel (2006)
Am J Physiol Endocrinol Metab
291, E611-E620
| Abstract »
| Full Text »
| PDF »
- Melanin Pigmentation in Mammalian Skin and Its Hormonal Regulation.
- A. Slominski, D. J. Tobin, S. Shibahara, and J. Wortsman (2004)
Physiol Rev
84, 1155-1228
| Abstract »
| Full Text »
| PDF »
- Endoplasmic Reticulum Stress Is a Determinant of Retrovirus-Induced Spongiform Neurodegeneration.
- D. E. Dimcheff, S. Askovic, A. H. Baker, C. Johnson-Fowler, and J. L. Portis (2003)
J. Virol.
77, 12617-12629
| Abstract »
| Full Text »
| PDF »
|
|
