C57BL/6NCya-Plnem1/Cya
Common Name
Pln-KO
Product ID
S-KO-03714
Backgroud
C57BL/6NCya
Strain ID
KOCMP-18821-Pln-B6N-VA
When using this mouse strain in a publication, please cite “Pln-KO Mouse (Catalog S-KO-03714) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
Basic Information
Strain Name
Pln-KO
Strain ID
KOCMP-18821-Pln-B6N-VA
Gene Name
Product ID
S-KO-03714
Gene Alias
Plb
Background
C57BL/6NCya
NCBI ID
Modification
Conventional knockout
Chromosome
Chr 10
Phenotype
Datasheet
Application
--
Strain Description
Ensembl Number
ENSMUST00000219491
NCBI RefSeq
NM_001141927
Target Region
Exon 3
Size of Effective Region
~0.2 kb
Overview of Gene Research
Pln, short for phospholamban, is a key modulator of Ca2+-homeostasis. It inhibits sarcoplasmic reticulum (SR) calcium-ATPase (SERCA2a), thereby regulating cardiac contractility. Calcium cycling regulated by Pln is crucial for normal heart function [2].
The p.Arg14del variant of the Pln gene (PLN-R14del) causes cardiomyopathy, leading to severe heart failure in humans. In a PLN-R14del mouse model, which mimics human disease characteristics, DWORF overexpression delays cardiac fibrosis, heart failure, and extends lifespan by reducing abnormal sarcoplasmic reticulum clusters. This indicates that abnormal S/ER organization in PLN-R14del cardiomyopathy contributes to cardiac dysfunction [1]. Additionally, in transgenic mice with the PLN-R14del+/- mutation, there is hyperdynamic Ca2+ handling, suggesting a loss of SERCA2a inhibition by PLN, and depressed energy metabolism, pointing to early metabolic stress involvement in the pathogenesis of this arrhythmogenic cardiomyopathy model [3].
In conclusion, Pln is essential for regulating calcium cycling and cardiac contractility. Studies using mouse models, especially those with the PLN-R14del mutation, have revealed its role in cardiomyopathy development. These models contribute significantly to understanding the mechanisms underlying heart failure and related cardiac diseases associated with Pln genetic variants.
References:
1. Stege, Nienke M, Eijgenraam, Tim R, Oliveira Nunes Teixeira, Vivian, de Boer, Rudolf A, Silljé, Herman H W. 2023. DWORF Extends Life Span in a PLN-R14del Cardiomyopathy Mouse Model by Reducing Abnormal Sarcoplasmic Reticulum Clusters. In Circulation research, 133, 1006-1021. doi:10.1161/CIRCRESAHA.123.323304. https://pubmed.ncbi.nlm.nih.gov/37955153/
2. Vafiadaki, Elizabeth, Haghighi, Kobra, Arvanitis, Demetrios A, Kranias, Evangelia G, Sanoudou, Despina. 2022. Aberrant PLN-R14del Protein Interactions Intensify SERCA2a Inhibition, Driving Impaired Ca2+ Handling and Arrhythmogenesis. In International journal of molecular sciences, 23, . doi:10.3390/ijms23136947. https://pubmed.ncbi.nlm.nih.gov/35805951/
3. Maniezzi, Claudia, Eskandr, Marem, Florindi, Chiara, Lodola, Francesco, Zaza, Antonio. 2024. Early consequences of the phospholamban mutation PLN-R14del+/- in a transgenic mouse model. In Acta physiologica (Oxford, England), 240, e14082. doi:10.1111/apha.14082. https://pubmed.ncbi.nlm.nih.gov/38214033/
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
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