title: Effect of diet on the survival and phenotype of a mouse model for spinal muscular atrophy.
abstract: Proximal spinal muscular atrophy (SMA) is a leading genetic cause of infant death. Patients with SMA lose alpha-motor neurons in the ventral horn of the spinal cord which leads to skeletal muscle weakness and atrophy. SMA is the result of reduction in Survival Motor Neuron (SMN) expression. Transgenic mouse models of SMA have been generated and are extremely useful in understanding the mechanisms of motor neuron degeneration in SMA and in developing new therapeutic candidates for SMA patients. Several research groups have reported varying average lifespans of SMNDelta7 SMA mice (SMN2(+/+);SMNDelta7(+/+);mSmn(-/-)), the most commonly used mouse model for preclinical therapeutic candidate testing. One environmental factor that varied between research groups was maternal diet. In this study, we compared the effects of two different commercially available rodent chows (PicoLab20 Mouse diet and Harlan-Teklad 22/5 diet) on the survival and motor phenotype of the SMNDelta7 mouse model of SMA. Specifically, the PicoLab20 diet significantly extends the average lifespan of the SMNDelta7 SMA mice by approximately 25% and improved the motor phenotype as compared to the Harlan diet. These findings indicate that maternal diet alone can have considerable impact on the SMA phenotype.
authors: Department of Molecular and Cellular Biochemistry, College of Medicine, The Ohio State University, Columbus, OH, USA. butchbach.1@osu.edu
authors: Matthew E R
authors: ME
authors: Butchbach
authors: Ferrill F
authors: FF
authors: Rose
authors: Sarah
authors: S
authors: Rhoades
authors: John
authors: J
authors: Marston
authors: John T
authors: JT
authors: McCrone
authors: Rachel
authors: R
authors: Sinnott
authors: Christian L
authors: CL
authors: Lorson
mesh_terms: D020155:3-Hydroxybutyric Acid / Q000097:blood; D000818:Animals; D001786:Blood Glucose / Q000032:analysis; D004032:Diet; D004195:Disease Models, Animal; D005260:Female; D008297:Male; D051379:Mice; D008822:Mice, Transgenic; D009134:Muscular Atrophy, Spinal / Q000178:diet therapy* / Q000401:mortality / Q000503:physiopathology*; D055533:Survival of Motor Neuron 1 Protein / Q000235:genetics
chemical_list: D001786:Blood Glucose; C526472:Smn1 protein, mouse; D055533:Survival of Motor Neuron 1 Protein; D020155:3-Hydroxybutyric Acid