A murine model for the del(GJB6-D13S1830) deletion recapitulating the phenotype of human DFNB1 hearing impairment: generation and functional and histopathological study

Table 2 Characteristics of DFNB1 mouse models

Model genotype	Gjb2^loxP/loxP Otog-Cre	Gjb2^loxP/loxP Foxg1-Cre or Pax2-Cre	Gjb2^loxP/loxP Rosa26^CreERT	Gjb2^loxP/loxP ROSA26^Cre/Esr1	Gjb2 c.35delG KI	Dfnb1^em274/em274
Type	CKO	CKO	TMX-inducible CKO	TMX-inducible CKO	KI	KO
Cochlear structures without Cx26	Organ of Corti	Organ of Corti & spiral limbus	Organ of Corti, KD elsewhere	Organ of Corti & spiral limbus	All	All
Cx30 expression	Normal	Normal	Normal	Normal	Reduced	Abolished
Hearing loss (ABR threshold in dB SPL)	Severe (70), all frequencies	Severe (80), all frequencies	Severe (80), all frequencies	Profound (> 110), all frequencies	Profound (> 80), all frequencies	Profound (> 90), all frequencies
Age at ABR	P30	P21 & P84	P21, P30, P60, P120	P14, P20, P30	P14 & P35	P30
Organ of Corti	Degeneration with missing supporting cells	Degeneration with missing supporting cells, no TC	Degeneration with missing supporting cells	Degeneration with missing supporting cells, no TC	Immature, no TC	Immature, no TC
Hair cells	Death of OHC, intact IHC	Death of OHC, intact IHC	Death of OHC, intact IHC	Not reported	Some OHC loss, intact IHC	Some OHC loss, intact IHC
References	Cohen-Salmon et al. 2002 [15]	Wang et al. 2009 [41] Chang et al. 2015 [10] Liang et al. 29	Sun et al. 2009 [37] Chen et al. 2014a [11] Chang et al. 2015 [10] Chen et al. 2018 [13]	Chen et al. 2014b [12] Zhu et al. 2015 [43]	Li et al. 2023 [28]	This work

Abbreviations: CKO Conditional knockout, KI knockin, KO knockout, TMX tamoxifen, KD knockdown, TC tunnel of Corti, OHC outer hair cells, IHC inner hair cells

ISSN: 1471-2164