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Col7a1 KO
产品名称:
Col7a1 KO
产品编号:
C001539
品系背景:
C57BL/6JCya
其他模型
* 使用本品系发表的文献需注明:Col7a1 KO mice (Catalog C001539) were purchased from Cyagen.
交付类型
周龄
性别
基因型
数量
品系描述
Col7a1 KO小鼠是利用基因编辑技术敲除人类COL7A1基因在小鼠体内的同源基因Col7a1所构建的营养不良性大疱性表皮松解症(DEB)研究模型,纯合Col7a1 KO小鼠缺少Col7a1基因和COL7A1蛋白的表达,在出生后第一天就出现前后脚掌皮肤的红肿和起泡症状,并在出生后3天内死亡。组织学检测结果显示,Col7a1 KO小鼠皮肤出现显著的皮下水肿,并存在表皮和真皮层分离现象,这与临床上人类营养不良性大疱性表皮松解症(DEB)致病机制和病理特征大致相同。因此,Col7a1 KO小鼠可用于营养不良性大疱性表皮松解症(DEB)的机制研究,以及治疗药物的研发、筛选和评价。
相关疾病:
Epidermolysis Bullosa Pruriginosa
Transient Bullous Dermolysis of the Newborn
Epidermolysis Bullosa Dystrophica, Autosomal Recessive
Nail Disorder, Nonsyndromic Congenital, 8
Epidermolysis Bullosa Dystrophica, Pretibial
Epidermolysis Bullosa Dystrophica, Autosomal Dominant
Epidermolysis Bullosa with Congenital Localized Absence of Skin and Deformity of Nails
Autosomal Dominant Generalized Dystrophic Epidermolysis Bullosa
Epidermolysis Bullosa Dystrophica
Recessive Dystrophic Epidermolysis Bullosa
Hyperpigmentation of the Skin
Autosomal Recessive Generalized Dystrophic Epidermolysis Bullosa, Severe Form
Localized Dystrophic Epidermolysis Bullosa, Pretibial Form
Localized Dystrophic Epidermolysis Bullosa, Nails Only
Localized Dystrophic Epidermolysis Bullosa, Acral Form
Autosomal Recessive Generalized Dystrophic Epidermolysis Bullosa, Intermediate Form
Skin Disease
Nail Disorder, Nonsyndromic Congenital, 4
Beckwith-Wiedemann Syndrome
Microcephaly
Thyroid Gland Disease
Epidermolytic Hyperkeratosis
Epidermolytic Hyperkeratosis 1
Ichthyosis
Nevus, Epidermal
Hepatoblastoma
应用领域
大疱性表皮松解症(EB)