C57BL/6JCya-Sdhaem1flox/Cya
Common Name
Sdha-flox
Product ID
S-CKO-13404
Backgroud
C57BL/6JCya
Strain ID
CKOCMP-66945-Sdha-B6J-VA
Status
When using this mouse strain in a publication, please cite “Sdha-flox Mouse (Catalog S-CKO-13404) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
The standard delivery applies for a guaranteed minimum of three heterozygous carriers. Breeding services for homozygous carriers and/or specified sex are available.
Basic Information
Strain Name
Sdha-flox
Strain ID
CKOCMP-66945-Sdha-B6J-VA
Gene Name
Product ID
S-CKO-13404
Gene Alias
FP, SDH2, SDHF, 4921513A11, 1500032O14Rik, 2310034D06Rik
Background
C57BL/6JCya
Gene Full Name
succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
Modification
Conditional knockout
NCBI ID
Phenotype
Chromosome
Chr 13 (Mouse)
Application
--
Datasheet
Strain Description
Ensembl Transcript ID
ENSMUST00000022062
NCBI Transcript ID
NM_023281
Target Region
Exon 4~6
Size of Effective Region
~3.7 kb
Overview of Gene Research
Sdha, short for succinate dehydrogenase subunit A, is a key component of the succinate dehydrogenase complex. This complex is involved in the tricarboxylic acid (TCA) cycle and mitochondrial respiratory chain, playing a crucial role in cellular energy metabolism [1,2,3,4,5,6,7].
Loss-of-function of the succinate dehydrogenase complex is seen in 20-40% of all KIT/PDGFRA-negative gastrointestinal stromal tumors (GISTs). Among SDH-deficient GISTs, SDHA mutations are the most common (30%), leading to loss of SDHA and SDHB protein expression [1]. SDHA pathogenic germline variants (PGVs) are identified in up to 10% of patients with paraganglioma and phaeochromocytoma and up to 30% with wild-type GISTs [2]. In phaeochromocytomas and paragangliomas, SDHA PVs account for up to 2.8% of cases, with tumors often presenting as single ones, commonly in the head and neck or abdomen, and a 25.5% metastasis rate [3]. In hepatocellular carcinoma, downregulation of SDHA/B is observed, promoting cancer proliferation by stabilizing YAP/TAZ [4]. In diabetic cardiomyopathy, CAV1 binds with SDHA, triggering its ubiquitination and degradation, resulting in mitochondrial dysfunction, which can be alleviated by LNT [6]. In primary antibody deficiency, SDHA gain-of-function leads to fumarate accumulation, engaging the KEAP1-Nrf2 system to drive inflammatory cytokine transcription [7].
In conclusion, Sdha is essential for normal cellular energy metabolism. Its dysregulation, as revealed through various disease-related studies rather than KO/CKO mouse models in the provided references, is associated with multiple diseases including GISTs, paraganglioma, phaeochromocytoma, hepatocellular carcinoma, diabetic cardiomyopathy, and primary antibody deficiency. Understanding Sdha's role in these conditions may provide insights for disease management and treatment.
References：
1. Schipani, Angela, Nannini, Margherita, Astolfi, Annalisa, Pantaleo, Maria A. 2023. SDHA Germline Mutations in SDH-Deficient GISTs: A Current Update. In Genes, 14, . doi:10.3390/genes14030646. https://pubmed.ncbi.nlm.nih.gov/36980917/
2. Hanson, Helen, Durkie, Miranda, Lalloo, Fiona, Woodward, Emma Roisin, Maher, Eamonn R. 2022. UK recommendations for SDHA germline genetic testing and surveillance in clinical practice. In Journal of medical genetics, 60, 107-111. doi:10.1136/jmedgenet-2021-108355. https://pubmed.ncbi.nlm.nih.gov/35260474/
3. Kaplan, Adam I, Dwight, Trisha, Luxford, Catherine, Benn, Diana E, Clifton-Bligh, Roderick J. 2024. SDHA-related phaeochromocytoma and paraganglioma: review and clinical management. In Endocrine-related cancer, 31, . doi:10.1530/ERC-24-0111. https://pubmed.ncbi.nlm.nih.gov/39133175/
4. Yuan, Tao, Zhou, Tianyi, Qian, Meijia, Zhu, Hong, Yang, Bo. 2022. SDHA/B reduction promotes hepatocellular carcinoma by facilitating the deNEDDylation of cullin1 and stabilizing YAP/TAZ. In Hepatology (Baltimore, Md.), 78, 103-119. doi:10.1002/hep.32621. https://pubmed.ncbi.nlm.nih.gov/35713976/
5. Bausch, Birke, Schiavi, Francesca, Ni, Ying, Eng, Charis, Neumann, Hartmut P H. . Clinical Characterization of the Pheochromocytoma and Paraganglioma Susceptibility Genes SDHA, TMEM127, MAX, and SDHAF2 for Gene-Informed Prevention. In JAMA oncology, 3, 1204-1212. doi:10.1001/jamaoncol.2017.0223. https://pubmed.ncbi.nlm.nih.gov/28384794/
6. Hu, Shuiqing, Luo, Jinlan, Guo, Ping, Fang, Qin, Wang, Yan. 2023. Lentinan alleviates diabetic cardiomyopathy by suppressing CAV1/SDHA-regulated mitochondrial dysfunction. In Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie, 167, 115645. doi:10.1016/j.biopha.2023.115645. https://pubmed.ncbi.nlm.nih.gov/37804808/
7. Burgener, Anne-Valérie, Bantug, Glenn R, Meyer, Benedikt J, Recher, Mike, Hess, Christoph. 2019. SDHA gain-of-function engages inflammatory mitochondrial retrograde signaling via KEAP1-Nrf2. In Nature immunology, 20, 1311-1321. doi:10.1038/s41590-019-0482-2. https://pubmed.ncbi.nlm.nih.gov/31527833/
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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