Table 2.

Col3a1 mouse models. Only original references are provided except in case whereby there is some debate regarding the nature of the mutation.

Model	Genotype-Method	Phenotype	Reference
Col3a1−/−	Targeted deletion Col3a1 promoter and exon 1	5% survival after birth, major skin lesions, vascular rupture, abnormal fibril organization.	[53]
Col3a1+/−		Mild phenotype, normal life span, aortic lesions, reduced collagen III, elevated MMP9 levels.	[55]
Col3a1+/− (Col3a1m1Lsmi/+)	Targeted deletion (Col3a1 promoter and exon 1–39)- in frame deletion exon 33–39 upon subsequent analysis.	Mild phenotype, aortic dissection, reduced collagen III. 30% lethality at 3 month	[56]
Col3a1Tg-G182S/+	Overexpression of Col3a1 transgene harbouring glycine substitution (Gly182Ser)	Thin and fragile skin, open wounds, vascular fragility, reduced collagen III, abnormal fibril organization	[57], [58]
Col3a1G209S/+	CRISPR glycine substitution	Vascular phenotype, sudden death due to aortic rupture, abnormal fibril organization, median survival 400 days	[51]
Col3a1G938D/+	CRISPR glycine substitution	Vascular phenotype, sudden death due to aortic rupture, abnormal fibril organization, median survival 45 days.	[51]
Tsk2/+ (Col3a1 C33S/+)	ENU mutagenesis, missense mutation in N-terminal propeptide	Tight skin (increased ECM deposition), thick collagen fibrils, model of systemic sclerosis	[60]