Mediators of a long-term movement abnormality in a Drosophila melanogaster model of classic galactosemia

DMM Disease Models and Mechanisms

Volumn 5, Issue 6, 2012, Pages 796-803

  Ryan, Emily L ^a   DuBoff, Brian ^b   Feany, Mel B ^b   Fridovich Keil, Judith L ^c  

^a EMORY UNIVERSITY   (United States)

^b BRIGHAM AND WOMEN S HOSPITAL   (United States)

^c EMORY UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

GALACTOSE; GLUCOSE; PHOSPHATE;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BRAIN TISSUE; DIET RESTRICTION; DIETARY INTAKE; DISEASE SEVERITY; DROSOPHILA MELANOGASTER; EMBRYO; ENZYME ACTIVITY; FLIGHT MUSCLE; GALACTOSEMIA; GENE EXPRESSION; HETEROZYGOTE; HISTOLOGY; HOMOZYGOTE; MALE; MOTOR DYSFUNCTION; MUSCLE TISSUE; MUTANT; NONHUMAN; NULL ALLELE; PRIORITY JOURNAL; PROGNOSIS; STARTLE REFLEX; SYNAPSE; TRANSGENE;

AGING; ANIMALS; BRAIN; DIET; DISEASE MODELS, ANIMAL; DROSOPHILA MELANOGASTER; DROSOPHILA PROTEINS; DYSKINESIAS; GALACTOSE; GALACTOSEMIAS; GALACTOSEPHOSPHATES; GALACTOSYLTRANSFERASES; HUMANS; MOTOR ACTIVITY; MOVEMENT; MUSCLES; STARTLE REACTION; TIME FACTORS;

DROSOPHILA MELANOGASTER;

EID: 84869998075     PISSN: 17548403     EISSN: 17548411     Source Type: Journal    
DOI: 10.1242/dmm.009050     Document Type: Article

References (16)

1. Akai, S. (1979). Genetic variation in walking ability of Drosophila melanogaster. Jpn. J. Genet. 54, 317-324.
2. Benzer, S. (1967). Behavioral mutants of Drosophila isolated by countercurrent distribution. Proc. Natl. Acad. Sci. USA 58, 1112-1119.
3. Beutler, E., Baluda, M. L., Sturgeon, P. and Day, R. W. (1965). A new genetic abnormality resulting in galactose-1-phosphate uridyltransferase deficiency. Lancet 1, 353-354.
4. Fridovich-Keil, J. L. and Walter, J. H. (2008). Galactosemia. In The Online Metabolic &Molecular Bases of Inherited Disease (ed. D. Valle, A. Beaudet, B. Vogelstein, K. Kinzler, S. Antonarakis and A. Ballabio), part 7, ch. 72. New York: McGraw Hill.
5. Ganetzky, B. and Wu, C. (1982). Indirect suppression involving behavioral mutants with altered nerve excitability in Drosophila melanogaster. Genetics 100, 597-614. (Pubitemid 12065377)
6. Gitzelmann, R. and Steinmann, B. (1984). Galactosemia: how does long-term treatment change the outcome? Enzyme 32, 37-46. (Pubitemid 14028074)
7. Kaufman, F. R., Xu, Y. K., Ng, W. G. and Donnell, G. N. (1988). Correlation of ovarian function with galactose-1-phosphate uridyl transferase levels in galactosemia. J. Pediatr. 112, 754-756.
8. Komrower, G. M. (1983). Clouds over galactosemia. Lancet 1, 190.
9. Kushner, R., Ryan, E., Sefton, J., Sanders, R., Lucioni, P., Moberg, K. and Fridovich-Keil, J. (2010). A Drosophila melanogaster model of classic galactosemia. Dis. Model. Mech. 3, 618-627.
10. Leslie, N. D., Yager, K. L., McNamara, P. D. and Segal, S. (1996). A mouse model of galactose-1-phosphate uridyl transferase deficiency. Biochem. Mol. Med. 59, 7-12. (Pubitemid 26346751)
11. Mellman, W. J. and Tedesco, T. A. (1965). An improved assay of erythrocyte and leukocyte galactose-1-phosphate uridyl transferase: stabilization of the enzyme by a thiol protective reagent. J. Lab. Clin. Med. 66, 980-986.
12. Rhodenizera, D., Martina, I., Bhandaria, P., Pletcherb, S. and Grotewiel, M. (2008). Genetic and environmental factors impact age-related impairment of negative geotaxis in Drosophila by altering age-dependent climbing speed. Exp. Gerontol. 43, 739-748.
13. Sanders, R., Sefton, J., Moberg, K. and Fridovich-Keil, J. (2010). UDP-galactose 4′epimerase (GALE) is essential for development of Drosophila melanogaster. Dis. Model. Mech. 3, 628-638.
14. Segal, S. (1989). Disorders of galactose metabolism. In The Metabolic Basis of Inherited Disease (ed. D. Scriver, A. Beaudet, W. Sly and D. Valle), pp. 453-480. New York: McGraw Hill.
15. Waggoner, D. D., Buist, N. R. and Donnell, G. N. (1990). Long-term prognosis in galactosaemia: results of a survey of 350 cases. J. Inherit. Metab. Dis. 13, 802-818.
16. Waisbren, S., Potter, N., Gordon, C., Green, R., Greenstein, P., Gubbels, C., Rubio-Gozalbo, E., Schomer, D., Welt, C., Anastasoaie, V. et al. (2012). The adult galactosemic phenotype. J. Inherit. Metab. Dis. 35, 279-286.