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C57BL/6JCya-Pccaem1flox/Cya
Common Name:
Pcca-flox
Product ID:
S-CKO-18304
Background:
C57BL/6JCya
Product Type
Age
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Sex
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Basic Information
Strain Name
Pcca-flox
Strain ID
CKOCMP-110821-Pcca-B6J-VB
Gene Name
Pcca
Product ID
S-CKO-18304
Gene Alias
-
Background
C57BL/6JCya
NCBI ID
110821
Modification
Conditional knockout
Chromosome
14
Phenotype
MGI:97499
Document
Click here to download >>
Application
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Rare Disease Data Center >>
Note
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Pccaem1flox/Cya mice (Catalog S-CKO-18304) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000038374
NCBI RefSeq
NM_144844
Target Region
Exon 9
Size of Effective Region
~1.4 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Pcca, the gene encoding the alpha subunit of propionyl-CoA carboxylase, is crucial for the function of this enzyme. Propionyl-CoA carboxylase is involved in the metabolism of several amino acids, odd-chain fatty acids, and cholesterol side-chains, playing a significant role in normal cellular metabolism [1].

A study showed that the PCCA c.1285-1416A>G variation activates a pseudoexon. This activation disrupts normal gene expression, leading to a deficiency of the propionyl-CoA carboxylase enzyme and causing the severe metabolic disorder propionic acidemia. However, blocking this region of regulation with splice-switching antisense oligonucleotides can restore normal splicing and rescue enzyme activity in patient fibroblasts and in a CRISPR-created cellular model. Moreover, blocking the inclusion of the non-activated wild-type pseudoexon can increase both PCCA and PCCB protein levels, enhancing the activity of the heterododecameric enzyme, which could be a potential treatment strategy for propionic acidemia [1].

In conclusion, Pcca is essential for the proper function of propionyl-CoA carboxylase in normal cellular metabolism. The discovery of the role of pseudoexon activation and the potential treatment strategy through splice-switching antisense oligonucleotides in Pcca-related propionic acidemia provides new insights into the treatment of this metabolic disorder.

References:
1. Spangsberg Petersen, Ulrika Simone, Dembic, Maja, Martínez-Pizarro, Ainhoa, Desviat, Lourdes Ruiz, Andresen, Brage Storstein. 2023. Regulating PCCA gene expression by modulation of pseudoexon splicing patterns to rescue enzyme activity in propionic acidemia. In Molecular therapy. Nucleic acids, 35, 102101. doi:10.1016/j.omtn.2023.102101. https://pubmed.ncbi.nlm.nih.gov/38204914/
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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