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Mouse Anti-DGUOK Recombinant Antibody (D0796) (V2LY-0425-LY256)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
D0796
Antibody Isotype
IgG1, κ
Application
WB, IP, IF, ELISA

Basic Information

Immunogen
Amino acids 191-277 mapping at the C-terminus of human DGUOK.
Host Species
Mouse
Specificity
Human
Antibody Isotype
IgG1, κ
Clonality
Monoclonal Antibody
Application Notes
ApplicationNote
WB1:100-1:1,000
IP1-2 µg per 100-500 µg of total protein (1 ml of cell lysate)
IF(ICC)1:50-1:500
ELISA1:100-1:1,000

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
Gelatin & PBS
Preservative
Sodium Azide
Concentration
0.2 mg/ml
Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
deoxyguanosine kinase
Entrez Gene ID
1716
UniProt ID
Q16854
Function
Phosphorylates deoxyguanosine and deoxyadenosine in the mitochondrial matrix, with the highest efficiency for deoxyguanosine (PubMed:8692979, PubMed:8706825, PubMed:11687801, PubMed:17073823, PubMed:23043144).

In non-replicating cells, where cytosolic dNTP synthesis is down-regulated, mtDNA synthesis depends solely on DGUOK and TK2. Phosphorylates certain nucleoside analogs (By similarity).

Widely used as target of antiviral and chemotherapeutic agents.
Biological Process
ATP biosynthetic process Source: Ensembl
dGTP metabolic process Source: Ensembl
Guanosine metabolic process Source: ProtInc
Mitochondrial ATP synthesis coupled electron transport Source: Ensembl
Negative regulation of neuron projection development Source: Ensembl
Protein phosphorylation Source: Ensembl
Purine-containing compound salvage Source: Reactome
Purine deoxyribonucleoside metabolic process Source: UniProtKB
Cellular Location
Mitochondrion
Involvement in disease
Mitochondrial DNA depletion syndrome 3 (MTDPS3):
A disorder due to mitochondrial dysfunction characterized by onset in infancy of progressive liver failure, hypoglycemia, increased lactate in body fluids, and neurologic abnormalities including hypotonia, encephalopathy, peripheral neuropathy. Affected tissues show both decreased activity of the mtDNA-encoded respiratory chain complexes and mtDNA depletion.
Portal hypertension, non-cirrhotic (NCPH):
An autosomal recessive disorder characterized by portal hypertension associated with hepatosplenomegaly, in absence of cirrhosis, extrahepatic diseases, and splanchnic venous thrombosis. Portal hypertension is defined by a portal venous system pressure that is at least 5 mm Hg higher than the pressure in the inferior vena cava. High pressure in the portal venous system leads to shunting of blood through vessels that are poorly suited to carrying large blood volumes, resulting in collateral circulation and splenomegaly. NCPH patients show normal liver function.
Progressive external ophthalmoplegia with mitochondrial DNA deletions, autosomal recessive 4 (PEOB4):
A form of progressive external ophthalmoplegia, a mitochondrial myopathy characterized by progressive paralysis of the levator palpebrae, orbicularis oculi, and extraocular muscles. PEOB4 patients manifest clinically variable features including mitochondrial myopathy with or without progressive external ophthalmoplegia, recurrent rhabdomyolysis, and adult-onset lower motor neuron syndrome with mild cognitive impairment.
More Infomation

Doğulu, N., Kırsaçlıoğlu, C. T., Köse, E., Aksu, A. Ü., Kuloğlu, Z., Kansu, A., & Eminoğlu, F. T. (2021). The clinical variations and diagnostic challenges of deoxyguanosine kinase deficiency: a descriptive case series. Journal of Pediatric Endocrinology and Metabolism, 34(10), 1341-1347.

Vanden Avond, M. A., Meng, H., Beatka, M. J., Helbling, D. C., Prom, M. J., Sutton, J. L., ... & Lawlor, M. W. (2021). The nucleotide prodrug CERC‐913 improves mtDNA content in primary hepatocytes from DGUOK‐deficient rats. Journal of Inherited Metabolic Disease, 44(2), 492-501.

Maines, E., & Iodice, A. (2021). Neurophthalmological Findings in Deoxyguanosine Kinase Deficiency: A Poor Outcome Predictor. Journal of Pediatric Neurology, 19(06), 457-458.

Wu, N., Song, H., Ren, Y., Tao, S., & Li, S. (2020). DGUOK‐AS1 promotes cell proliferation in cervical cancer via acting as a ceRNA of miR‐653‐5p. Cell Biochemistry and Function, 38(7), 870-879.

Zhou, X., Curbo, S., Zhao, Q., Krishnan, S., Kuiper, R., & Karlsson, A. (2019). Severe mtDNA depletion and dependency on catabolic lipid metabolism in DGUOK knockout mice. Human molecular genetics, 28(17), 2874-2884.

Lin, S., Huang, C., Sun, J., Bollt, O., Wang, X., Martine, E., ... & Yang, S. (2019). The mitochondrial deoxyguanosine kinase is required for cancer cell stemness in lung adenocarcinoma. EMBO molecular medicine, 11(12), e10849.

Waich, S., Roscher, A., Brunner-Krainz, M., Cortina, G., Köstl, G., Feichtinger, R. G., ... & Vodopiutz, J. (2019). Severe Deoxyguanosine Kinase Deficiency in Austria: A 6-Patient Series. Journal of pediatric gastroenterology and nutrition, 68(1), e1-e6.

Munro, B., Horvath, R., & Müller, J. S. (2019). Nucleoside supplementation modulates mitochondrial DNA copy number in the dguok−/− zebrafish. Human molecular genetics, 28(5), 796-803.

Fang, W., Song, P., Xie, X., Wang, J., Lu, Y., Li, G., & Abuduxikuer, K. (2017). A fatal case of mitochondrial DNA depletion syndrome with novel compound heterozygous variants in the deoxyguanosine kinase gene. Oncotarget, 8(48), 84309.

Ünal, Ö., Hişmi, B., Kılıç, M., Gülşen, H. H., Coşkun, T., Sivri, S. H., ... & Tokatlı, A. (2017). Deoxyguanosine kinase deficiency: a report of four patients. Journal of Pediatric Endocrinology and Metabolism, 30(6), 697-702.

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For research use only. Not intended for any clinical use.

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