智鼠故事 
B6-hLMNA
复苏/繁育服务
HUGO-GT®全基因组人源化模型
* 使用本品系发表的文献需注明：B6-hLMNA mice (Catalog I001124) were purchased from Cyagen.
交付类型
周龄
性别
基因型
数量
只
品系描述
本模型为小鼠Lmna基因人源化模型，将小鼠Lmna基因替换为包含3'UTR区域的人源LMNA基因，可用于研究神经肌肉性疾病、心脏病、早衰综合症等疾病的致病机制和治疗药物的临床前评价。该模型纯合子是可存活且可育的。
相关疾病：
Hutchinson-Gilford Progeria Syndrome
Muscular Dystrophy, Congenital, Lmna-Related
Mandibuloacral Dysplasia with Type a Lipodystrophy
Emery-Dreifuss Muscular Dystrophy 3, Autosomal Recessive
Cardiomyopathy, Dilated, with Hypergonadotropic Hypogonadism
Lipodystrophy, Familial Partial, Type 2
Charcot-Marie-Tooth Disease, Axonal, Type 2b1
Cardiomyopathy, Dilated, 1a
Emery-Dreifuss Muscular Dystrophy 2, Autosomal Dominant
Heart-Hand Syndrome, Slovenian Type
Restrictive Dermopathy 2
Dilated Cardiomyopathy
Restrictive Dermopathy
Familial Partial Lipodystrophy
Laminopathy
Cardiomyopathy, Dilated, 1h
Autosomal Recessive Axonal Hereditary Motor and Sensory Neuropathy
Atypical Werner Syndrome
Lmna-Related Cardiocutaneous Progeria Syndrome
Autosomal Semi-Dominant Severe Lipodystrophic Laminopathy
Muscular Dystrophy
Emery-Dreifuss Muscular Dystrophy
Restrictive Dermopathy 1
Emery-Dreifuss Muscular Dystrophy 1, X-Linked
Autosomal Recessive Limb-Girdle Muscular Dystrophy Type 2b
Microtia
Left Ventricular Noncompaction
Neuromuscular Disease
Cardiomyopathy, Dilated, 1b
Brugada Syndrome
Monogenic Diabetes
Cardiomyopathy, Dilated, 1e
Arrhythmogenic Right Ventricular Cardiomyopathy
Charcot-Marie-Tooth Disease
Charcot-Marie-Tooth Disease, Axonal, Type 2e
Muscular Dystrophy, Limb-Girdle, Autosomal Recessive 2
Familial Isolated Arrhythmogenic Ventricular Dysplasia, Biventricular Form
Familial Isolated Arrhythmogenic Ventricular Dysplasia, Left Dominant Form
Familial Isolated Arrhythmogenic Ventricular Dysplasia, Right Dominant Form
Hypercholesterolemia, Familial, 1
Cardiomyopathy, Familial Hypertrophic, 1
Long Qt Syndrome
Familial Isolated Dilated Cardiomyopathy
Lipodystrophy, Familial Partial, Type 1
Myocarditis
Brugada Syndrome 5
Arrhythmogenic Right Ventricular Dysplasia, Familial, 9
Cardiac Conduction Defect
Peripheral Nervous System Disease
应用领域
早衰综合征（Hutchinson-Gilford progeria syndrome，HGPS）
