C57BL/6JCya-Ndufab1em1/Cya
Common Name:
Ndufab1-KO
Product ID:
S-KO-18171
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
Price:
Contact for Pricing
Basic Information
Strain Name
Ndufab1-KO
Strain ID
KOCMP-70316-Ndufab1-B6J-VB
Gene Name
Product ID
S-KO-18171
Gene Alias
2210401F17Rik; 2310039H15Rik; 2610003B19Rik; 8kDa; 9130423F15Rik; ACP; CI-SDAP; SDAP
Background
C57BL/6JCya
NCBI ID
Modification
Conventional knockout
Chromosome
7
Phenotype
Document
Application
--
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Ndufab1em1/Cya mice (Catalog S-KO-18171) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000033157
NCBI RefSeq
NM_028177
Target Region
Exon 3~4
Size of Effective Region
~2.1 kb
Detailed Document
Overview of Gene Research
Ndufab1, also known as mitochondrial acyl carrier protein, is a nuclear-encoded subunit of complex I of the mitochondrial respiratory chain. It plays a crucial role in mitochondrial bioenergetics, coordinating the assembly of respiratory complexes I, II, and III, as well as supercomplexes. It is involved in regulating iron-sulfur biosynthesis and complex I subunit stability, and is essential for mitochondrial energy and ROS metabolism [1,3,4].
Cardiac-specific deletion of Ndufab1 in mice led to defective bioenergetics, elevated ROS levels, progressive dilated cardiomyopathy, 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].
In conclusion, Ndufab1 is a key regulator of mitochondrial metabolism. Gene-knockout mouse models have revealed its significance in preventing heart-related diseases like heart failure, as well as metabolic disorders such as obesity and insulin resistance. Understanding Ndufab1's functions provides potential therapeutic targets for these diseases.
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