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
Krit1ECKO, Krit1BECKO	VE-Cadherin-CreERT2; Krit1fl/fl or Slco1c1(BAC)-Cre; Krit1fl/fl	Numerous lesions in the hindbrain	University of Pennsylvania	Mixed
Ccm2ECKO	VE-Cadherin-CreERT2; Ccm2fl/fl	Same as Krit1(B)ECKO	Centenary Institute	Mixed
N/A	PDGFb-CreERT2; Pdcd10fl/−	Same as Krit1(B)ECKO	Duke University	C57BL/6J
Krit1ECKO; Adamts4−/−	VE-Cadherin-CreERT2; Krit1fl/fl; Adamts4−/−	No reduction in lesion formation	University of Pennsylvania	Mixed
Krit1ECKO; Adamts1fl/−	VE-Cadherin-CreERT2; Krit1fl/fl; Adamts1fl/−	No reduction in lesion formation	University of Pennsylvania	Mixed
Krit1ECKO; Adamts1fl/fl; Adamts4−/−	VE-Cadherin-CreERT2; Krit1fl/fl; Adamts1fl/fl; Adamts4−/−	No reduction in lesion formation	University of Pennsylvania	Mixed
Ccm2ECKO; Adamts5−/−	VE-Cadherin-CreERT2; Ccm2fl/fl; Adamts5−/−	Significant reduction in lesion formation	Centenary Institute	Mixed
Krit1ECKO; Adamts5fl/fl;	VE-Cadherin-CreERT2; Krit1fl/fl; Adamts5fl/fl;	Significant reduction in lesion formation	University of Pennsylvania	Mixed
ADAMTS5BEC-GOF + doxycycline	Slco1c1(BAC)-Cre; LSL-rtTA; tetO-Adamts5	Nascent CCM-like vascular changes	University of Pennsylvania	Mixed
Krit1BECKO + doxycycline	Slco1c1(BAC)-Cre; Krit1fl/fl	Significant reduction in lesion formation	University of Pennsylvania	Mixed
Krit1BECKO; ADAMTS5BEC-GOF + doxycycline	Slco1c1(BAC)-Cre; Krit1fl/fl; LSL-rtTA; tetO-Adamts5	Increase in lesion formation	University of Pennsylvania	Mixed
Krit1ECKO; VcanRC/+	VE-Cadherin-CreERT2; Krit1fl/fl; VcanRC/+	Significant reduction in lesion formation	University of Pennsylvania	Mixed