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C57BL/6JCya-Naxdem1flox/Cya
Common Name:
Naxd-flox
Product ID:
S-CKO-14459
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Naxd-flox
Strain ID
CKOCMP-69225-Naxd-B6J-VA
Gene Name
Naxd
Product ID
S-CKO-14459
Gene Alias
0710008K08Rik; 2810407E01Rik; Carkd
Background
C57BL/6JCya
NCBI ID
69225
Modification
Conditional knockout
Chromosome
8
Phenotype
MGI:1913353
Document
Click here to download >>
Application
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Rare Disease Data Center >>
Note
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Naxdem1flox/Cya mice (Catalog S-CKO-14459) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000033901
NCBI RefSeq
NM_026995
Target Region
Exon 2
Size of Effective Region
~2.0 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Naxd, or NAD(P)HX dehydratase, is a highly conserved enzyme essential for the intracellular repair of redox-inactive derivatives NAD(P)HX, which are formed from the damage of central cofactors NAD(P)H by hydration [1,3,5]. This repair process is crucial for maintaining normal cellular metabolism and function. The gene plays a significant role in metabolic pathways related to the maintenance of NAD(P)H homeostasis. Genetic models are valuable for studying Naxd as they can help reveal its precise function in vivo.

Pathogenic variants in Naxd are associated with a metabolite repair disorder. Naxd-deficiency patients show distinct clinical features depending on the sub-cellular compartment affected by the enzymatic deficiency. Variants affecting both cytosolic and mitochondrial isoforms present with neurological defects, seizures, and skin lesions, while those affecting only the mitochondrial isoform present with myopathy, moderate neuropathy, and cardiac issues without skin lesions, seizures, or neurological degeneration [1]. Additionally, Naxd-deficiency can lead to progressive neurological deterioration, often associated with febrile illness, along with skin lesions, elevated lactate levels, and brain neuroimaging abnormalities [3]. In some cases, niacin-based therapies have shown promise in improving outcomes and normalizing metabolic abnormalities [2,4].

In conclusion, Naxd is essential for the repair of damaged NAD(P)HX, thus maintaining normal cellular metabolism. Studies on Naxd-deficiency, often through patient-based genetic analysis (since no KO/CKO mouse models were mentioned in the provided references), have revealed its role in various disease-related phenotypes, mainly neurological and metabolic disorders. Understanding Naxd function is crucial for the early identification of related diseases and the development of more effective therapeutic interventions.

References:
1. Van Bergen, Nicole J, Walvekar, Adhish S, Patraskaki, Myrto, Linster, Carole L, Christodoulou, John. 2022. Clinical and biochemical distinctions for a metabolite repair disorder caused by NAXD or NAXE deficiency. In Journal of inherited metabolic disease, 45, 1028-1038. doi:10.1002/jimd.12541. https://pubmed.ncbi.nlm.nih.gov/35866541/
2. Manor, Joshua, Calame, Daniel G, Gijavanekar, Charul, Scaglia, Fernando, Elsea, Sarah H. . Niacin therapy improves outcome and normalizes metabolic abnormalities in an NAXD-deficient patient. In Brain : a journal of neurology, 145, e36-e40. doi:10.1093/brain/awac065. https://pubmed.ncbi.nlm.nih.gov/35231119/
3. Majethia, Purvi, Mishra, Shivani, Rao, Lakshmi Priya, Rao, Raghavendra, Shukla, Anju. 2021. NAD(P)HX dehydratase (NAXD) deficiency due to a novel biallelic missense variant and review of literature. In European journal of medical genetics, 64, 104266. doi:10.1016/j.ejmg.2021.104266. https://pubmed.ncbi.nlm.nih.gov/34161859/
4. Van Bergen, Nicole J, Gunanayagam, Karen, Bournazos, Adam M, Cooper, Sandra T, Christodoulou, John. 2023. Severe NAD(P)HX Dehydratase (NAXD) Neurometabolic Syndrome May Present in Adulthood after Mild Head Trauma. In International journal of molecular sciences, 24, . doi:10.3390/ijms24043582. https://pubmed.ncbi.nlm.nih.gov/36834994/
5. Van Bergen, Nicole J, Guo, Yiran, Rankin, Julia, Ellard, Sian, Hakonarson, Hakon. . NAD(P)HX dehydratase (NAXD) deficiency: a novel neurodegenerative disorder exacerbated by febrile illnesses. In Brain : a journal of neurology, 142, 50-58. doi:10.1093/brain/awy310. https://pubmed.ncbi.nlm.nih.gov/30576410/
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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