FVB-Pcca KO/hPCCA*A138T
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Basic Information
Model Name
FVB-Pcca KO/hPCCA*A138T
Product ID
C001583
Background
FVB/NJCya
Gene Alias
--
Classification
Metabolic Disease Models,Rare Disease Models
Chromosome
14
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Note: When using this mouse strain in a publication, please cite “FVB-Pcca KO/hPCCA*A138T mice (Catalog C001583) were purchased from Cyagen.”
Strain Description
This strain is a propionic acidemia (PA) disease model generated in the FVB/NJCya background strain using gene editing technology to replace a portion of the mouse Pcca gene sequence with the coding sequence (CDS) of the human PCCA gene carrying the A138T mutation. This model, by simultaneously knocking out the endogenous mouse Pcca gene and expressing the mutant human PCCA gene, mimics the metabolic characteristics of human PA patients and is suitable for investigating the pathogenic mechanisms and therapeutic interventions for propionic acidemia.
Application Area
Investigation of the pathogenic mechanisms and therapeutic interventions for propionic acidemia (PA)
Validation Data