Spastic mouse

Kingsmore SF, Giros B, Suh D, Bieniarz M, Caron MG, Seldin MF. Glycine receptor beta-subunit gene mutation in spastic mouse associated with LINE-1 element insertion. Nat Genet 1994 7 136-141. 

Pelizaeus-Merzbacher disease (classical and connatal forms) and spastic paraplegia X-linked PLP Variable hypomyelination due to different mutations in the major structural protein of CNS myelin similar to rodent mutants such as the jimpy mouse 1,23... 

Furthermore, two autosomal recessive mouse mutants, spastic (spa) and spasmodic (spd), exhibit identical phenotypes with striking similarities to STHE in human. Phenotypically normal at birth, the affected animals develop rigidity and fall in response to sudden tactile or acoustic stimuli after the second postnatal week. A lethal mutant, oscillator (ot), displays more severe symptoms and may be allelic to spa. 

Properties Colorless liq. clove balsamic sharp spicy sweet odor m.w. 166.22 sp.gr. 1.038 dens. 8.621-8.633 Ib/gal b.p. 125-126 C (14 mm Hg) flash pt. > 110 C ref. index 1.5243-1.5257 (20 C) Toxicology LD50 (oral, rat) 2600 mg/kg, (IP, mouse) 150 mg/kg, (subcut, mouse) 1450 mg/kg, (skin, rabbit) 310 mg/kg toxic toxic by inh., ing., skin contact vapor/mist irritating to eyes, mucous membranes, upper respiratory tract skin irritant readily absorbed thru skin may cause spastic paralysis TSCA listed Precaution Incompat. with strong oxidizing agents... 

C flash pt. (OC) 135 C ref. index 1.5065 Toxicology LD50 (oral, rat) 4920 mg/kg, (IP, mouse) 100 mg/kg, (skin, rabbit) > 5 ml/kg poison by IP route mod. toxic by ing. primary irritant skin, eye, and respiratory system irritant may cause somnolence, muscle weakness or spasticity TSCA listed Precaution Combustible exposed to heat or flame reacts vigorously with oxidizers Hazardous Decomp. Prods. Heated to decomp., emits toxic fumes of NOx Uses Polyurethane catalyst for elastomers and foam... 

Toxicology LD50 (oral, rat) 2700 mg/kg, (IP, mouse) 514 mg/kg harmful by ing. may be harmful by inh. or skin absorp. irritating to eyes, skin, mucous membranes, upper respiratory tract can be absorbed thru skin may cause muscle contraction/spasticity in rats, inh. has caused... 

---