Phenotypes Associated with This Genotype

Genotype
MGI:3577344

• Allelic Composition: inad/inad
• Genetic Background: C57BL/6-inad

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

Cross section of brain of inad/inad mouse.

mortality/aging

premature death ( J:97670 )

• died usually 3-8 weeks after birth

nervous system

abnormal nervous system morphology ( J:97670 )

• neurodystrophic changes with numerous spheroid bodies in nervous tissues such as in the hippocampus, thalamus, hypothalamus, pons, callosal body, striatum, globus pallidus, and the cervical, thoracic and lumbar vertebrae

dilated fourth ventricle ( J:97670 )

dilated third ventricle ( J:97670 )

abnormal medulla oblongata morphology ( J:97670 )

• axonal dystrophic change represented by spheroid bodies in the gracile tract of the medulla oblongata

small hippocampus ( J:97670 )

• hippocampus is hardly distinguishable due to its thinness and caudal displacement caused by ventricular enlargement

axonal dystrophy ( J:97670 )

behavior/neurological

limb grasping ( J:97670 )

• when suspended by tail, the limbs become flexed and then fold toward the trunk compared to controls in which limbs are held extended out from the trunk

ataxia ( J:97670 )

• sensory ataxia

hindlimb paralysis ( J:97670 )

• progressive motor paralysis and atrophy of the hindlimbs, which show muscle weakness with foot-droop

paraparesis ( J:97670 )

• dragging of hindlimbs

limbs/digits/tail

abnormal hindlimb morphology ( J:97670 )

• initially mutants exhibit an abnormal limb posture and later show atrophy of the hindlimbs

adipose tissue

decreased total body fat amount ( J:97670 )

• lean body, particularly in the lower half

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
neurodegeneration with brain iron accumulation 2a		DOID:0110735	OMIM:256600	J:97670