B6-hNRL Mouse
Product Name
B6-hNRL Mouse
Product ID
C001799
Strain Name
C57BL/6JCya-Nrltm1(hNRL)/Cya
Backgroud
C57BL/6JCya
Status
When using this mouse strain in a publication, please cite “B6-hNRL Mouse (Catalog C001799) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
Gene Name
NRL
Gene Alias
RP27, D14S46E, NRL-MAF
NCBI ID
Chromosome
Chr 14 (Human)
MGI ID
Datasheet
Strain Description
The NRL (neural retina leucine zipper) gene encodes a basic motif-leucine zipper (bZIP) transcription factor of the Maf subfamily, which plays a critical role in the development and function of photoreceptor cells, particularly rods, in the mammalian retina. Gene expression of NRL is highly specific to the retina, appearing in postmitotic neuronal cells during embryonic development and maintaining high levels in mature neural retina. It functions as a master regulator of rod photoreceptor cell fate, working in conjunction with other transcription factors like CRX and NR2E3 to activate rod-specific genes (e.g., rhodopsin) and repress cone-specific genes. Cellular tissues predominantly labeled by NRL include rod photoreceptor nuclei, with some labeling also observed in rod and cone inner segments, somata, and synapses, and weak labeling in the cytoplasm of scattered cells in the inner nuclear and ganglion cell layers. Mutations in the NRL gene are associated with various inherited retinal degenerative diseases, most notably Retinitis Pigmentosa (RP), which can manifest as autosomal dominant (Retinitis Pigmentosa 27) or autosomal recessive forms (clumped pigmentary retinal degeneration, resembling Enhanced S-cone Syndrome), leading to progressive loss of vision [1-3].
The B6-hNRL mouse is a humanized model, constructed by replacing the sequences from 5'UTR to 3'UTR of the endogenous mouse Nrl gene with the corresponding human NRL gene sequence. B6-hNRL mice can be used for research into the pathogenesis of various inherited retinal degenerative diseases such as Retinitis Pigmentosa (RP). They are also useful for the screening, development, and safety evaluation of NRL-targeted drugs.
Reference
Kim JW, Yang HJ, Brooks MJ, Zelinger L, Karakülah G, Gotoh N, Boleda A, Gieser L, Giuste F, Whitaker DT, Walton A, Villasmil R, Barb JJ, Munson PJ, Kaya KD, Chaitankar V, Cogliati T, Swaroop A. NRL-Regulated Transcriptome Dynamics of Developing Rod Photoreceptors. Cell Rep. 2016 Nov 22;17(9):2460-2473.
Liang X, Brooks MJ, Swaroop A. Developmental genome-wide occupancy analysis of bZIP transcription factor NRL uncovers the role of c-Jun in early differentiation of rod photoreceptors in the mammalian retina. Hum Mol Genet. 2022 Nov 10;31(22):3914-3933.
Moore SM, Skowronska-Krawczyk D, Chao DL. Targeting of the NRL Pathway as a Therapeutic Strategy to Treat Retinitis Pigmentosa. J Clin Med. 2020 Jul 13;9(7):2224.
Strain Strategy
Figure 1. Gene editing strategy of B6-hNRL Mice. The sequences from 5'UTR to 3'UTR of the endogenous mouse Nrl gene will be replaced with the sequences from 5'UTR to 3'UTR of the human NRL gene.
Application Area
NRL-targeted drug screening, development, and evaluation;
Research on the pathological mechanisms and therapeutic approaches of various inherited retinal degenerative diseases such as Retinitis Pigmentosa (RP).
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