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C57BL/6JCya-Ndufa9em1flox/Cya
Common Name:
Ndufa9-flox
Product ID:
S-CKO-18111
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Ndufa9-flox
Strain ID
CKOCMP-66108-Ndufa9-B6J-VB
Gene Name
Ndufa9
Product ID
S-CKO-18111
Gene Alias
1010001N11Rik
Background
C57BL/6JCya
NCBI ID
66108
Modification
Conditional knockout
Chromosome
6
Phenotype
MGI:1913358
Document
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Application
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Rare Disease Data Center >>
Note
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Ndufa9em1flox/Cya mice (Catalog S-CKO-18111) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000205002
NCBI RefSeq
NM_025358
Target Region
Exon 2~3
Size of Effective Region
~1.4 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Ndufa9, or NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, is closely associated with the activity and function of mitochondrial respiratory chain complex I [1]. Mitochondrial respiratory chain complex I consists of 44 different subunits and 3 functional modules, with Ndufa9 being a Q-module subunit required for complex I assembly or stability [2]. The gene plays a crucial role in cellular energy production via oxidative phosphorylation and is essential for normal mitochondrial function.

In mice, Ndufa9 promotes mitochondrial respiration, thermogenesis, and the browning of white adipose tissue. When Ndufa9 was studied in white fat browning model mice, it was found to enhance mitochondrial function, mitochondrial complex I activity, ATP synthesis, and mitochondrial respiration, all of which contribute to the browning process [1]. In addition, gene knockout using transcription activator-like effector nucleases (TALENs) in HEK293T cells showed that loss of Ndufa9 led to impaired assembly of complex I, with cells unable to grow in galactose medium. Re-expression of Ndufa9 restored the defects in complex I assembly, indicating its role in stabilizing the junction between membrane and matrix arms of complex I, a late and critical step for complex I biogenesis and activity [3].

Mutations in Ndufa9 have also been linked to Leigh syndrome. For example, via exome sequencing, a novel homozygous Ndufa9 missense variant was identified in a patient with childhood-onset progressive generalized dystonia and axonal peripheral neuropathy. Complex I assembly analysis in patient fibroblasts showed reduced complex I abundance and an accumulation of Q-module subassemblies, with the severity of the clinical phenotype correlating with the severity of the effects of the Ndufa9 variant on complex I assembly [2]. A pathogenic mutation in Ndufa9 was also found in a Kurdish patient with neonatally fatal Leigh syndrome and complex I deficiency, and lentiviral transduction with wild-type Ndufa9 restored complex I activity in the patient's fibroblasts [4].

In conclusion, Ndufa9 is essential for mitochondrial respiratory chain complex I assembly and function. Studies using mouse models and gene knockout experiments have revealed its role in processes like white adipose tissue browning and its association with Leigh syndrome. These findings provide important insights into the mechanisms of energy metabolism-related biological processes and mitochondrial-associated diseases, potentially guiding future research and treatment strategies in these areas.

References:
1. Liu, Yuexia, Liu, Zunhai, Ren, Zeyu, Qiu, Guiping, Sun, Chao. 2024. NDUFA9 and its crotonylation modification promote browning of white adipocytes by activating mitochondrial function in mice. In The international journal of biochemistry & cell biology, 171, 106583. doi:10.1016/j.biocel.2024.106583. https://pubmed.ncbi.nlm.nih.gov/38657899/
2. Baertling, F, Sánchez-Caballero, L, van den Brand, M A M, Rodenburg, R J T, Nijtmans, L G J. 2017. NDUFA9 point mutations cause a variable mitochondrial complex I assembly defect. In Clinical genetics, 93, 111-118. doi:10.1111/cge.13089. https://pubmed.ncbi.nlm.nih.gov/28671271/
3. Stroud, David A, Formosa, Luke E, Wijeyeratne, Xiaonan W, Nguyen, Thanh N, Ryan, Michael T. 2012. Gene knockout using transcription activator-like effector nucleases (TALENs) reveals that human NDUFA9 protein is essential for stabilizing the junction between membrane and matrix arms of complex I. In The Journal of biological chemistry, 288, 1685-90. doi:10.1074/jbc.C112.436766. https://pubmed.ncbi.nlm.nih.gov/23223238/
4. van den Bosch, B J C, Gerards, M, Sluiter, W, de Coo, I F M, Smeets, H J M. 2011. Defective NDUFA9 as a novel cause of neonatally fatal complex I disease. In Journal of medical genetics, 49, 10-5. doi:10.1136/jmedgenet-2011-100466. https://pubmed.ncbi.nlm.nih.gov/22114105/
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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