mortality/aging
• mice fed a thiamine-restricted diet (0.60 mg thiamine/100 g food) die within 30 days of starting the diet
• mice fed a thiamine-restricted diet with an even lower percentage of thiamine (0.27 mg thiamine/100 g food) die within 18 days
• however, mice fed a conventional diet containing 1.71 mg thiamine/100 g of food survive for over 6 months without any disease phenotype
• mice that are returned to a conventional diet after 14 days on a thiamine-restricted diet show no disease phenotypes
|
nervous system
astrocytosis
(
J:246593
)
• astrocyte activation is increased in the bilateral thalamic area of mice fed with a thiamine-restricted diet for 14 days
|
• after 14 days of a thiamine-restricted diet, the numbers of NeuN+ neurons in the submedial nucleus of the thalamus and the dorsal area of the thalamus are decreased to approximately 30%
• after 14 days of an even lower thiamine-restricted diet, the numbers of NeuN+ neurons are hardly detected in the thalamic area, including the submedial nucleus of the thalamus and ventral anterior-lateral complex of the thalamus
|
behavior/neurological
• mice fed a thiamine-restricted diet exhibit immobility
|
homeostasis/metabolism
• thiamine levels in the blood of mice fed a conventional diet are deceased at 7 weeks of age
• thiamine levels in the blood of mice fed a thiamine-restricted diet are decreased
• thiamine concentration in the brain of mice fed a conventional diet is deceased
• thiamine concentration in the brain decreases steadily in mice fed a thiamine-restricted diet before mice present disease phenotypes
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
biotin-responsive basal ganglia disease | DOID:0050659 |
OMIM:607483 |
J:246593 |