Life Span Body Weight Age of Onset and Reproductive Capacity

The ChAc-model mice are viable and exhibit normal life span. There was no significant difference in body weight at the age of 87 weeks. However, at older ages (>70 weeks) they displayed disturbances in motor function and erythrocyte morphological changes, similar to the features of adult onset and gradual progression seen in humans with ChAc [8]. Although as far as we know human male ChAc patients show normal reproductive capacity, our male ChAc-model mice that carried homozygous mutation were totally infertile. Immunoblot analysis demonstrated the most abundant chorein expression in mouse testis, and

Table 1 Phenotype of ChAc-model mouse [6, 10]

Characteristic

<70 weeks

>70 weeks

Wild-type versus ChAc-model

Reproductive capacity

Male Infertile

Female Fertile

Life span

Body weight loss n.s.

Hematological test Osmotic fragility test

Behavioral tests Foot print test

Stride length n.s.

Rotarod test

Latency to fall n.s.

Open field test

Moving distance n.s.

Social interaction

Contact time n.s.

Neurotransmitter analysis HVA (mid brain)

NE, DA, 5-HT, 5-HIAA, and GABA Brain pathology

Weight ratio of striatum/whole brain -No. of TUNEL-positive cells -

GFAP staining -

Others Gephyrin

Immunoblotting

Striatum -

Hippocampus -

Immunostaining -

GABAa receptor al subunit Immunoblotting

Striatum -

Hippocampus -

Immunostaining -

GABAA receptor y2 subunit Immunoblotting

Striatum -

Hippocampus -

Immunostaining -

Wild-type < ChAc-model mouse*

Wild-type > ChAc-model mouse*1

Wild-type > ChAc-model mouse*

Wild-type > ChAc-model mouse*

Wild-type > ChAc-model mouse*

Wild-type > ChAc-model mouse* n.s.

Wild-type > ChAc-model mouse* Wild-type < ChAc-model mouse* Wild-type < ChAc-model mouse

Wild-type < ChAc-model mouse* Wild-type < ChAc-model mouse* Wild-type < ChAc-model mouse n.s. n.s.

Wild-type < ChAc-model mouse

Wild-type < ChAc-model mouse* Wild-type < ChAc-model mouse* Wild-type < ChAc-model mouse

5-HIAA, 5-hydroxyindoleacetic acid; 5-HT, 5-hydroxytyramine; DA, dopamine; GABA, gamma aminobutyric acid; GFAP, glial fibrillary acidic protein; HVA, homovanillic acid; NE, norepinephrine; n.s., not significant; TUNEL, Tdt-mediated dUTP nick-end labeling

immunohistochemically, variable chorein-like immunoreactivities were observed in the stages of differentiation of sperm [5]. These suggest that chorein in mouse testis may have an important role in spermatogenesis.

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