C57BL/6JCya-Ndufab1em1flox/Cya
Common Name:
Ndufab1-flox
Product ID:
S-CKO-14779
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
Price:
Contact for Pricing
Basic Information
Strain Name
Ndufab1-flox
Strain ID
CKOCMP-70316-Ndufab1-B6J-VA
Gene Name
Product ID
S-CKO-14779
Gene Alias
2210401F17Rik; 2310039H15Rik; 2610003B19Rik; 8kDa; 9130423F15Rik; ACP; CI-SDAP; SDAP
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
7
Phenotype
Document
Application
--
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Ndufab1em1flox/Cya mice (Catalog S-CKO-14779) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000033157
NCBI RefSeq
NM_028177
Target Region
Exon 3~4
Size of Effective Region
~2.6 kb
Detailed Document
Overview of Gene Research
NDUFAB1, also known as mitochondrial acyl carrier protein, is a crucial component in mitochondrial metabolism. It is a subunit of complex I in the mitochondrial respiratory chain and is involved in coordinating the assembly of respiratory complexes I, II, and III, as well as supercomplexes. This coordination occurs through regulating iron-sulfur biosynthesis and complex I subunit stability, thus playing a significant role in mitochondrial bioenergetics and related pathways [1,3,4].
In mouse models, cardiac-specific deletion of Ndufab1 led to defective bioenergetics, elevated ROS levels, progressive dilated cardiomyopathy, and eventually heart failure and sudden death [1]. Skeletal muscle-specific ablation of NDUFAB1 caused systemic disruption of glucose homeostasis, defective insulin signaling, growth arrest, and early death within 5 postnatal days [2]. These gene knockout (KO) mouse models clearly demonstrate the essential role of Ndufab1 in maintaining normal physiological functions related to energy metabolism and metabolic regulation.
In conclusion, Ndufab1 is essential for mitochondrial energy and ROS metabolism by coordinating the assembly of respiratory complexes and supercomplexes. The KO mouse models have revealed its significance in preventing heart failure, obesity, and insulin resistance, highlighting its potential as a therapeutic target in these disease areas [1,2].
References:
1. Hou, Tingting, Zhang, Rufeng, Jian, Chongshu, Cheng, Heping, Wang, Xianhua. 2019. NDUFAB1 confers cardio-protection by enhancing mitochondrial bioenergetics through coordination of respiratory complex and supercomplex assembly. In Cell research, 29, 754-766. doi:10.1038/s41422-019-0208-x. https://pubmed.ncbi.nlm.nih.gov/31366990/
2. Zhang, Rufeng, Hou, Tingting, Cheng, Heping, Wang, Xianhua. 2019. NDUFAB1 protects against obesity and insulin resistance by enhancing mitochondrial metabolism. In FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 33, 13310-13322. doi:10.1096/fj.201901117RR. https://pubmed.ncbi.nlm.nih.gov/31530015/
3. Triepels, R, Smeitink, J, Loeffen, J, Trijbels, F, van den Heuvel, L. . The human nuclear-encoded acyl carrier subunit (NDUFAB1) of the mitochondrial complex I in human pathology. In Journal of inherited metabolic disease, 22, 163-73. doi:. https://pubmed.ncbi.nlm.nih.gov/10234612/
4. Dibley, Marris G, Formosa, Luke E, Lyu, Baobei, Stroud, David A, Ryan, Michael T. 2019. The Mitochondrial Acyl-carrier Protein Interaction Network Highlights Important Roles for LYRM Family Members in Complex I and Mitoribosome Assembly. In Molecular & cellular proteomics : MCP, 19, 65-77. doi:10.1074/mcp.RA119.001784. https://pubmed.ncbi.nlm.nih.gov/31666358/
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