Mouse Genome Informatics
hm
    Btdtm1Bwol/Btdtm1Bwol
B6.Cg-Btdtm1Bwol
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
behavior/neurological
• mice fed a biotin-deprived diet exhibit behavioral and neurological symptoms unlike similarly treated wild-type mice
• by day 14 in mice fed a biotin-depleted diet
• however, biotin-treatment rescues tremors
• by day 14, mice fed a biotin-depleted diet exhibit tail and limb weakness unlike similarly treated wild-type mice
• by day 16, mice fed a biotin-depleted diet exhibit limb weakness to paralysis unlike similarly treated wild-type mice
• by day 14 in mice fed a biotin-depleted diet
• by day 14 in mice fed a biotin-depleted diet
• by day 14 in mice fed a biotin-depleted diet
• by day 16 in mice fed a biotin-depleted diet
• by day 16 in mice fed a biotin-depleted diet

integument
• by day 14, mice fed a biotin-depleted diet exhibit whitening of the fur around the eyes and subsequent graying of the fur over the entire body unlike similarly treated wild-type mice
• in mice fed a biotin-depleted diet
• however, treatment with biotin restores coat

homeostasis/metabolism
• in mice fed a biotin-depleted diet
• mice fed a biotin-depleted diet exhibit increased urinary biotin and biotin metabolites and 3-hydroxyisovaleric acid (20-fold) compared with similarly treated wild-type mice

growth/size
• in mice fed a biotin-depleted diet
• however, biotin treatment reverses weight loss

skeleton
• in mice fed a biotin-depleted diet

renal/urinary system
• mice fed a biotin-depleted diet exhibit increased urinary biotin and biotin metabolites and 3-hydroxyisovaleric acid (20-fold) compared with similarly treated wild-type mice

muscle
• in mice fed a biotin-depleted diet

vision/eye
• mice fed a biotin-depleted diet exhibit a difficult time keeping eyelids open unlike similarly treated wild-type mice

pigmentation
• by day 14, mice fed a biotin-depleted diet exhibit whitening of the fur around the eyes and subsequent graying of the fur over the entire body unlike similarly treated wild-type mice

Mouse Models of Human Disease
OMIM IDRef(s)
Biotinidase Deficiency 253260 J:168391