Expression of GC mRNA and enzyme in RecNciI and L444P mice. (A) A Northern blot with 5 μg of brain poly(A)+ RNA from a RecNciI homozygote mouse, a L444P homozygote mouse, a L444P heterozygote mouse, and a wild-type mouse. The blot first was probed with a mouse GC cDNA. After stripping, it was reprobed with a mouse metaxin cDNA. Finally, it was stripped once more and probed with a human actin cDNA. (B) Acid β-glucosidase activity was determined in extracts of liver, brain, and skin from homozygote L444P and RecNciI mice. Tissues from three mice of each genotype were assayed. The assays were repeated three times. The activity (±SD) is expressed as nmol substrate cleaved/hr per mg of protein.