C57BL/6JCya-Lrpap1em1/Cya
Common Name:
Lrpap1-KO
Product ID:
S-KO-16885
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
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Basic Information
Strain Name
Lrpap1-KO
Strain ID
KOCMP-16976-Lrpap1-B6J-VB
Gene Name
Product ID
S-KO-16885
Gene Alias
HBP44; RAP
Background
C57BL/6JCya
NCBI ID
Modification
Conventional knockout
Chromosome
5
Phenotype
Document
Application
--
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Lrpap1em1/Cya mice (Catalog S-KO-16885) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000030986
NCBI RefSeq
NM_013587
Target Region
Exon 2
Size of Effective Region
~0.6 kb
Detailed Document
Overview of Gene Research
Lrpap1, also known as receptor associated protein (RAP), is an endoplasmic reticulum chaperone and inhibitor of LDL receptor related protein 1 (LRP1) and related receptors. It is involved in ensuring proper formation of intermolecular disulfide bonds during LDL receptor biogenesis and escorts receptors through the endoplasmic reticulum and early Golgi compartment to prevent premature ligand-receptor interaction [4]. It has been associated with various biological processes and diseases, playing a role in lipid metabolism, immune response, and more. Genetic models such as knockout models can be valuable for further understanding its functions.
In zebrafish, lrpap1 deficiency leads to myopia through TGF-β-induced apoptosis. Homozygous mutant zebrafish showed myopic phenotypes with changes in eye axial length, scleral collagen fiber structure, and activation of apoptosis signaling [1]. In mouse microglial cell lines, activated or stressed microglia release LRPAP1, which can inhibit microglial phagocytosis and amyloid beta aggregation [2]. In humans, frameshift mutations in LRPAP1 are responsible for autosomal recessive high myopia, and childhood myopic foveoschisis can develop in those with LRPAP1-associated myopia [1,3].
In conclusion, Lrpap1 is crucial for multiple biological processes. Its deficiency in zebrafish models reveals its role in ocular refractive development and myopia. In microglial cell models, its release and function in regulating microglial activities and amyloid-beta pathology are demonstrated. These model-based studies contribute to understanding diseases related to myopia and neurodegeneration associated with amyloid-beta aggregation.
References:
1. Liu, Shanshan, Chen, Ting, Chen, Binghao, Lu, Xiaohe, Li, Jiali. 2022. Lrpap1 deficiency leads to myopia through TGF-β-induced apoptosis in zebrafish. In Cell communication and signaling : CCS, 20, 162. doi:10.1186/s12964-022-00970-9. https://pubmed.ncbi.nlm.nih.gov/36261846/
2. Reid, Kyle M, Brown, Guy C. 2023. LRPAP1 is released from activated microglia and inhibits microglial phagocytosis and amyloid beta aggregation. In Frontiers in immunology, 14, 1286474. doi:10.3389/fimmu.2023.1286474. https://pubmed.ncbi.nlm.nih.gov/38035103/
3. Almazyead, Mohammad, Alfakhri, Abdullmajeed S, Alsulaiman, Sulaiman M. 2024. Childhood Myopic Foveoschisis in LRPAP1-associated Myopia. In Middle East African journal of ophthalmology, 30, 182-184. doi:10.4103/meajo.meajo_151_23. https://pubmed.ncbi.nlm.nih.gov/39444998/
4. Neuhaus, Herbert, Gaul, Florian, Hollemann, Thomas. . lrpap1 as a specific marker of proximal pronephric kidney tubuli in Xenopus laevis embryos. In The International journal of developmental biology, 62, 319-324. doi:10.1387/ijdb.170295hn. https://pubmed.ncbi.nlm.nih.gov/29877571/
Quality Control Standard
Sperm Test
Pre-cryopreservation: Measurement of sperm concentration, determination of sperm viability.
Post-cryopreservation: A vial of cryopreserved sperms is selected for in-vitro fertilization from each batch.
Environmental Standards:SPF
Available Region:Global
Source:Cyagen