mortality/aging
• homozygotes usually die between 2 and 4 weeks of age
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endocrine/exocrine glands
• enlarged goblet cells
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growth/size/body
• at >P3, homozygotes display a 75% reduction in body weight relative to wild-type mice
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• at P3 or later, homozygotes are growth retarded relative to wild-type littermates
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behavior/neurological
nervous system
• type II afferent fibers lacking at birth and first post natal week in the medial and apical turns of the cochlea but they are present in the basal turn
• at age 19 days the pattern is reversed
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• homozygotes exhibit a loss of neurons in the dorsal root ganglia
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• homozygotes exhibit a loss of neurons in the dorsal root ganglia
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• frequency distributions of posttetanic synaptic enhancements indicate that in homozygotes most potentiations are close to 100% vs >150% in wild-type mice
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• after tetanic stimulation, homozygotes exhibit significantly reduced mean field EPSP slopes relative to wild-type mice, even when only successful LTPs are included
• in contrast, paired pulse facilitation (ratio of second EPSP slope to first EPSP slope) is unaffected
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• homozygotes show almost no LTP in the CA1 region of the hippocampus until P16 (only 1 successful LTP in 19 slices; 5%)
• from P17 to P28, the % of successful LTPs rises to 32.6%, but the magnitude is significantly smaller than in wild-type mice and declines slowly over time
• notably, general hippocampus anatomy and hippocampal pyramidal neuron morphology and pharmacology appear to be normal
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digestive/alimentary system
• generally hypotrophic
• mucosal layers of ileum and duodenum are normal
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• mucosal atrophy
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• enlarged goblet cells
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hearing/vestibular/ear
• type II afferent fibers lacking at birth and first post natal week in the medial and apical turns of the cochlea but they are present in the basal turn
• at age 19 days the pattern is reversed
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integument
• after 22 days, most hair follicles in later stages of regression (catagen) while controls are at the end of catagen or in the resting phase (telogen)
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cellular
• enlarged goblet cells
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