C57BL/6JCya-Tafazzinem1flox/Cya
Common Name:
Tafazzin-flox
Product ID:
S-CKO-13323
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
Price:
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Basic Information
Strain Name
Tafazzin-flox
Strain ID
CKOCMP-66826-Tafazzin-B6J-VA
Gene Name
Product ID
S-CKO-13323
Gene Alias
5031411C02Rik; 9130012G04Rik; G4.5; Taz
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
X
Phenotype
Document
Application
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Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Tafazzinem1flox/Cya mice (Catalog S-CKO-13323) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000114180
NCBI RefSeq
NM_001173547
Target Region
Exon 5~10
Size of Effective Region
~2.4 kb
Detailed Document
Overview of Gene Research
Tafazzin is a mitochondrial enzyme that exchanges fatty acids between phospholipids by phospholipid-lysophospholipid transacylation. Its most important in vivo substrate is cardiolipin, a mitochondria-specific lipid. This reaction alters lipid molecular species composition and physical properties. Tafazzin is crucial for mitochondrial function, and its mutations cause Barth syndrome [1,2].
A tafazzin knockdown (TAZKD) mouse model, a type of gene knockout model, has been used to study Barth syndrome. In this model, the physiological, biochemical, and ultrastructural abnormalities mirror those in Barth patients. Analyzing the TAZ-KD mouse hearts revealed significant lipid composition differences compared to wild-type, indicating genotype-dependent alterations in many lipid species. These findings offer potential avenues for BTHS diagnosis and treatment. Also, the peroxisome proliferator-activated receptor pan-agonist bezafibrate was suggested as a potential therapy as it ameliorated cardiomyopathy in TAZKD mice, increasing mitochondrial biogenesis [3,4].
In conclusion, Tafazzin is essential for mitochondrial lipid remodeling, especially of cardiolipin. The study of Tafazzin using gene knockout mouse models, like the TAZKD mouse, has provided crucial insights into the pathogenesis of Barth syndrome and potential therapeutic targets, which may also be relevant to understanding pediatric heart failure.
References:
1. Schlame, Michael, Xu, Yang. 2020. The Function of Tafazzin, a Mitochondrial Phospholipid-Lysophospholipid Acyltransferase. In Journal of molecular biology, 432, 5043-5051. doi:10.1016/j.jmb.2020.03.026. https://pubmed.ncbi.nlm.nih.gov/32234310/
2. Houtkooper, Riekelt H, Turkenburg, Marjolein, Poll-The, Bwee Tien, Kulik, Willem, Vaz, Frédéric M. 2009. The enigmatic role of tafazzin in cardiolipin metabolism. In Biochimica et biophysica acta, 1788, 2003-14. doi:10.1016/j.bbamem.2009.07.009. https://pubmed.ncbi.nlm.nih.gov/19619503/
3. Ren, Mindong, Miller, Paighton C, Schlame, Michael, Phoon, Colin K L. 2019. A critical appraisal of the tafazzin knockdown mouse model of Barth syndrome: what have we learned about pathogenesis and potential treatments? In American journal of physiology. Heart and circulatory physiology, 317, H1183-H1193. doi:10.1152/ajpheart.00504.2019. https://pubmed.ncbi.nlm.nih.gov/31603701/
4. Hachmann, Malte, Gülcan, Güntas, Rajendran, Ranjithkumar, Dudek, Jan, Karnati, Srikanth. 2024. Tafazzin deficiency causes substantial remodeling in the lipidome of a mouse model of Barth Syndrome cardiomyopathy. In Frontiers in molecular medicine, 4, 1389456. doi:10.3389/fmmed.2024.1389456. https://pubmed.ncbi.nlm.nih.gov/39086433/
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