Table 1. Summary of described phenotypes... | Rockefeller University Press

Table 1.

Summary of described phenotypes arising from inactivation/activation of ADAMTS genes or versican in the CCM mouse model

Abbreviated name	Genotype	Phenotype	Vivarium	Strain
Krit1^ECKO, Krit1^BECKO	VE-Cadherin-Cre^ERT2; Krit1^fl/fl or Slco1c1(BAC)-Cre; Krit1^fl/fl	Numerous lesions in the hindbrain	University of Pennsylvania	Mixed
Ccm2^ECKO	VE-Cadherin-Cre^ERT2; Ccm2^fl/fl	Same as Krit1^(B)ECKO	Centenary Institute	Mixed
N/A	PDGFb-Cre^ERT2; Pdcd10^fl/−	Same as Krit1^(B)ECKO	Duke University	C57BL/6J
Krit1^ECKO; Adamts4^−/−	VE-Cadherin-Cre^ERT2; Krit1^fl/fl; Adamts4^−/−	No reduction in lesion formation	University of Pennsylvania	Mixed
Krit1^ECKO; Adamts1^fl/−	VE-Cadherin-Cre^ERT2; Krit1^fl/fl; Adamts1^fl/−	No reduction in lesion formation	University of Pennsylvania	Mixed
Krit1^ECKO; Adamts1^fl/fl; Adamts4^−/−	VE-Cadherin-Cre^ERT2; Krit1^fl/fl; Adamts1^fl/fl; Adamts4^−/−	No reduction in lesion formation	University of Pennsylvania	Mixed
Ccm2^ECKO; Adamts5^−/−	VE-Cadherin-Cre^ERT2; Ccm2^fl/fl; Adamts5^−/−	Significant reduction in lesion formation	Centenary Institute	Mixed
Krit1^ECKO; Adamts5^fl/fl;	VE-Cadherin-Cre^ERT2; Krit1^fl/fl; Adamts5^fl/fl;	Significant reduction in lesion formation	University of Pennsylvania	Mixed
ADAMTS5^BEC-GOF + doxycycline	Slco1c1(BAC)-Cre; LSL-rtTA; tetO-Adamts5	Nascent CCM-like vascular changes	University of Pennsylvania	Mixed
Krit1^BECKO + doxycycline	Slco1c1(BAC)-Cre; Krit1^fl/fl	Significant reduction in lesion formation	University of Pennsylvania	Mixed
Krit1^BECKO; ADAMTS5^BEC-GOF + doxycycline	Slco1c1(BAC)-Cre; Krit1^fl/fl; LSL-rtTA; tetO-Adamts5	Increase in lesion formation	University of Pennsylvania	Mixed
Krit1^ECKO; Vcan^RC/+	VE-Cadherin-Cre^ERT2; Krit1^fl/fl; Vcan^RC/+	Significant reduction in lesion formation	University of Pennsylvania	Mixed

Abbreviated name	Genotype	Phenotype	Vivarium	Strain
Krit1^ECKO, Krit1^BECKO	VE-Cadherin-Cre^ERT2; Krit1^fl/fl or Slco1c1(BAC)-Cre; Krit1^fl/fl	Numerous lesions in the hindbrain	University of Pennsylvania	Mixed
Ccm2^ECKO	VE-Cadherin-Cre^ERT2; Ccm2^fl/fl	Same as Krit1^(B)ECKO	Centenary Institute	Mixed
N/A	PDGFb-Cre^ERT2; Pdcd10^fl/−	Same as Krit1^(B)ECKO	Duke University	C57BL/6J
Krit1^ECKO; Adamts4^−/−	VE-Cadherin-Cre^ERT2; Krit1^fl/fl; Adamts4^−/−	No reduction in lesion formation	University of Pennsylvania	Mixed
Krit1^ECKO; Adamts1^fl/−	VE-Cadherin-Cre^ERT2; Krit1^fl/fl; Adamts1^fl/−	No reduction in lesion formation	University of Pennsylvania	Mixed
Krit1^ECKO; Adamts1^fl/fl; Adamts4^−/−	VE-Cadherin-Cre^ERT2; Krit1^fl/fl; Adamts1^fl/fl; Adamts4^−/−	No reduction in lesion formation	University of Pennsylvania	Mixed
Ccm2^ECKO; Adamts5^−/−	VE-Cadherin-Cre^ERT2; Ccm2^fl/fl; Adamts5^−/−	Significant reduction in lesion formation	Centenary Institute	Mixed
Krit1^ECKO; Adamts5^fl/fl;	VE-Cadherin-Cre^ERT2; Krit1^fl/fl; Adamts5^fl/fl;	Significant reduction in lesion formation	University of Pennsylvania	Mixed
ADAMTS5^BEC-GOF + doxycycline	Slco1c1(BAC)-Cre; LSL-rtTA; tetO-Adamts5	Nascent CCM-like vascular changes	University of Pennsylvania	Mixed
Krit1^BECKO + doxycycline	Slco1c1(BAC)-Cre; Krit1^fl/fl	Significant reduction in lesion formation	University of Pennsylvania	Mixed
Krit1^BECKO; ADAMTS5^BEC-GOF + doxycycline	Slco1c1(BAC)-Cre; Krit1^fl/fl; LSL-rtTA; tetO-Adamts5	Increase in lesion formation	University of Pennsylvania	Mixed
Krit1^ECKO; Vcan^RC/+	VE-Cadherin-Cre^ERT2; Krit1^fl/fl; Vcan^RC/+	Significant reduction in lesion formation	University of Pennsylvania	Mixed