Biology Reference
In-Depth Information
pattern of synapses is altered on outer hair cells, with a single large termi-
nal instead of many small endings on each hair cell (Vetter et al. 1999).
The efferent supply of outer hair cells is believed to permit suppression
of cochlear responses, and this physiological feature is absent in the a9
mutants (Vetter et al. 1999).
Several neurological mutants show specific defects of cell types within
the central auditory pathways. The nervous, lurcher, Purkinje cell degener-
ation, and staggerer mutants all have specific cartwheel cell defects in the
dorsal cochlear nucleus, and in reeler mutants the dorsal cochlear nucleus
is disorganized with reduced numbers of granule cells (Berrebi and
Mugnaini 1988; Berrebi et al. 1990; Martin 1981).
TABLE 8.7. Mouse mutants with central auditory system defects
Key
Mutant name
Symbol
Gene
Chromosome
Inheritance
Origin
references
GATA binding
Gata3
Gata3
2 (7)
R
T
1
protein 3
Osteopetrosis,
op
,
Csf1
Csf1
3 (51)
R
S
2
Colony
Csfm
stimulating
factor 1 KO
Maloney sarcoma
Mos
Mos
4 (0)
SD
T
3, 4
oncogene KO
Pax5
KO
Pax5
,
BSAP
Pax5
4 (20.7)
SD
T
5, 6
Reeler
Reln
,
rl
Reln
5 (8)
R
S, C, I
7-9
Homeobox A1 KO
Hoxa1
Hoxa1
6 (26.3)
R, E
T
10-14
Lurcher
Lc
,
Grid1
,
Grid1
6 (29.6)
SD
S
15,16
cpr
,
ho
Deafwaddler,
dfw
,
wri
,
Atp2b2
6 (49.5)
R, SD
S
17-19
Wriggle mouse
Pmca2
,
Sagami,
Pmca
Atp2b2
2
KO
Quivering
qv
,
dyn
,
lnd
NK
7 (14.5)
R
S
20-23
Albino
c
,
Ty r
Ty r
7 (44)
R
S, R
24-26
Nervous
nr
NK
8 (8)
R
S
27
Myodystrophy
myd
,
fg
NK
8 (33)
R
S
28
Staggerer
sg
,
Rora
Rora
9 (36)
R
S
15,29
Foxb1a
KO
Fkh5
,
Mf3
,
Foxb1a
9 (41)
SD
T
30
Foxb1a
,
Tw h
Orthodenticle
Otx1
Otx1
11 (12)
R, E
T
31-34
homologue 1 KO
Trembler
Tr
,
Pmp22
Pmp22
11 (34.5)
D, SD
S
35-37
Homeobox B1 KO
Hoxb1
Hoxb1
11 (56)
R, E
T
38-41
Hypothyroidism
hyt
,
pet
,
Tshr
12 (37)
R
S
42-44
Tshr
Purkinje cell
pcd
NK
13 (37)
R
S
15
degeneration
Wabbler-lethal
wl
NK
14
R
S
45
