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Crb1-KO Mouse
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Crb1-KO Mouse
Product Name
Crb1-KO Mouse
Product ID
C001886
Strain Name
C57BL/6JCya-Crb1em1/Cya
Backgroud
C57BL/6JCya
Status
Live Mouse
When using this mouse strain in a publication, please cite “Crb1-KO Mouse (Catalog C001886) were purchased from Cyagen.”
Disease Animal Models
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The standard delivery applies for a guaranteed minimum of three heterozygous carriers. Breeding services for homozygous carriers and/or specified sex are available.
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Disease Animal Models
Basic Information
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Basic Information
Gene Name
Crb1
Gene Alias
CRB1-A, CRB1-B, CRB1-C, CRB1-A2, 7530426H14Rik, A930008G09Rik
NCBI ID
170788
Chromosome
Chr 1
MGI ID
MGI:2136343
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Datasheet
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Strain Description
The Crb1 gene, whose full name is Crumbs homolog 1, is a gene closely related to retinal development and function. The protein encoded by the Crb1 gene plays an important role in maintaining the connection and polarity between retinal pigment epithelial (RPE) cells and photoreceptor cells. RPE cells are responsible for absorbing and transmitting light signals, while photoreceptor cells are responsible for converting these signals into electrical signals, thus producing vision. Mutations in the Crb1 gene can lead to retinal dysplasia and functional disorders, causing a series of inherited retinal diseases, including Leber congenital amaurosis (LCA), retinitis pigmentosa (RP), and cone-rod dystrophy, etc. [1]. Retinal diseases caused by Crb1 gene mutations are highly heterogeneous, and different mutations can lead to different clinical manifestations and disease severities. Diseases caused by Crb1 gene mutations are mainly characterized by the damage and death of retinal pigment epithelial cells and photoreceptor cells, resulting in vision loss and blindness. In addition, Crb1 gene mutations may also be associated with other eye diseases such as abnormal eye development and myopia [2].
Crb1-KO mice are a gene knockout (KO) model in which exon 3 of the Crb1 gene in mice has been knocked out using gene-editing technology. This model can be used for studying the pathogenic mechanisms of retinal diseases such as Leber congenital amaurosis (LCA), retinitis pigmentosa (RP), and cone-rod dystrophy, as well as for the development of relevant treatment methods.
Reference
Boon N, Wijnholds J, Pellissier LP. Research Models and Gene Augmentation Therapy for CRB1 Retinal Dystrophies. Front Neurosci. 2020 Aug 14;14:860.
Ehrenberg M, Pierce EA, Cox GF, Fulton AB. CRB1: one gene, many phenotypes. Semin Ophthalmol. 2013 Sep-Nov;28(5-6):397-405.
Strain Strategy
The mouse Crb1 gene contains 12 exons. The ATG start codon is located in exon 1, and the TAG stop codon is located in exon 12. In this strain, the region of exon 3 has been knocked out using gene editing technology.
Figure 1. Diagram of the gene editing strategy for the generation of Crb1-KO mice.
Figure 1. Diagram of the gene editing strategy for the generation of Crb1-KO mice.
Application Area
Research on the pathogenic mechanism and related treatment methods of Leber congenital amaurosis (LCA);
Research on the pathogenic mechanism and related treatment methods of retinitis pigmentosa (RP);
Research on the pathogenic mechanism and related treatment methods of cone-rod dystrophy (CORD).
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