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Mouse Anti-HK1 Recombinant Antibody (CBFYH-1223) (CBMAB-H2194-FY)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBFYH-1223
Antibody Isotype
IgG2a
Application
WB, IHC-P, FC, IF

Basic Information

Specificity
Human
Antibody Isotype
IgG2a
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

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

Format
Liquid
Concentration
0.5 mg/mL
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
hexokinase 1
Introduction
Hexokinases phosphorylate glucose to produce glucose-6-phosphate, the first step in most glucose metabolism pathways. This gene encodes a ubiquitous form of hexokinase which localizes to the outer membrane of mitochondria. Mutations in this gene have been associated with hemolytic anemia due to hexokinase deficiency. Alternative splicing of this gene results in several transcript variants which encode different isoforms, some of which are tissue-specific.
Entrez Gene ID
3098
UniProt ID
P19367
Alternative Names
Hexokinase 1; Brain Form Hexokinase; Hexokinase Type I; EC 2.7.1.1; Glycolytic Enzyme; Hexokinase IR; Hexokinase-1; Hexokinase; EC 2.7.1; HK1-Ta
Function
Catalyzes the phosphorylation of various hexoses, such as D-glucose, D-glucosamine, D-fructose, D-mannose and 2-deoxy-D-glucose, to hexose 6-phosphate (D-glucose 6-phosphate, D-glucosamine 6-phosphate, D-fructose 6-phosphate, D-mannose 6-phosphate and 2-deoxy-D-glucose 6-phosphate, respectively) (PubMed:1637300, PubMed:25316723, PubMed:27374331).

Does not phosphorylate N-acetyl-D-glucosamine (PubMed:27374331).

Mediates the initial step of glycolysis by catalyzing phosphorylation of D-glucose to D-glucose 6-phosphate (By similarity).

Involved in innate immunity and inflammation by acting as a pattern recognition receptor for bacterial peptidoglycan (PubMed:27374331).

When released in the cytosol, N-acetyl-D-glucosamine component of bacterial peptidoglycan inhibits the hexokinase activity of HK1 and causes its dissociation from mitochondrial outer membrane, thereby activating the NLRP3 inflammasome (PubMed:27374331).
Biological Process
Canonical glycolysis Source: Reactome
Carbohydrate phosphorylation Source: CAFA
Cellular glucose homeostasis Source: GO_Central
Establishment of protein localization to mitochondrion Source: ParkinsonsUK-UCL
Fructose 6-phosphate metabolic process Source: UniProtKB
Glucose 6-phosphate metabolic process Source: UniProtKB
Glycolytic process Source: GO_Central
Inflammatory response Source: UniProtKB-KW
Innate immune response Source: UniProtKB-KW
Maintenance of protein location in mitochondrion Source: ParkinsonsUK-UCL
Mannose metabolic process Source: UniProtKB
Positive regulation of cytokine production involved in immune response Source: Ensembl
Positive regulation of interleukin-1 beta production Source: Ensembl
Cellular Location
Mitochondrion outer membrane; Cytosol. The mitochondrial-binding peptide (MBP) region promotes association with the mitochondrial outer membrane (Probable). Dissociates from the mitochondrial outer membrane following inhibition by N-acetyl-D-glucosamine, leading to relocation to the cytosol (PubMed:27374331).
Involvement in disease
Hexokinase deficiency (HK deficiency):
Rare autosomal recessive disease with nonspherocytic hemolytic anemia as the predominant clinical feature.
Neuropathy, hereditary motor and sensory, Russe type (HMSNR):
An autosomal recessive progressive complex peripheral neuropathy characterized by onset in the first decade of distal lower limb weakness and muscle atrophy resulting in walking difficulties. Distal impairment of the upper limbs usually occurs later, as does proximal lower limb weakness. There is distal sensory impairment, with pes cavus and areflexia. Laboratory studies suggest that it is a myelinopathy resulting in reduced nerve conduction velocities in the demyelinating range as well as a length-dependent axonopathy.
Retinitis pigmentosa 79 (RP79):
A form of retinitis pigmentosa, a retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. RP79 inheritance is autosomal dominant.
Neurodevelopmental disorder with visual defects and brain anomalies (NEDVIBA):
A disorder characterized by global developmental delay, speech delay, intellectual disability, structural brain abnormalities, and visual impairments including retinitis pigmentosa and optic atrophy.
More Infomation

Peretz, R. H., Zein, W. M., Hufnagel, R. B., Ku, C., Godfrey, R., Wolfe, L., ... & Toro, C. (2023). A de novo hexokinase 1 (HK1) variant presenting as Boucher–Neuhäuser syndrome. American Journal of Medical Genetics Part A, 191(2), 624-629.

Sundaram, S. M., Doughty, L. A., & Sereda, M. W. (2022). Location matters: hexokinase 1 in glucose metabolism and inflammation. Trends in Endocrinology & Metabolism.

De Jesus, A., Keyhani-Nejad, F., Pusec, C. M., Goodman, L., Geier, J. A., Stoolman, J. S., ... & Ardehali, H. (2022). Hexokinase 1 cellular localization regulates the metabolic fate of glucose. Molecular cell, 82(7), 1261-1277.

Šimčíková, D., Gardáš, D., Hložková, K., Hruda, M., Žáček, P., Rob, L., & Heneberg, P. (2021). Loss of hexokinase 1 sensitizes ovarian cancer to high-dose metformin. Cancer & Metabolism, 9(1), 1-18.

Li, Y., Tian, H., Luo, H., Fu, J., Jiao, Y., & Li, Y. (2020). Prognostic significance and related mechanisms of hexokinase 1 in ovarian cancer. OncoTargets and therapy, 11583-11594.

Reig, A., Norman, G. L., Garcia, M., Shums, Z., Ruiz-Gaspà, S., Bentow, C., ... & Pares, A. (2020). Novel anti–hexokinase 1 antibodies are associated with poor prognosis in patients with primary biliary cholangitis. Official journal of the American College of Gastroenterology| ACG, 115(10), 1634-1641.

Xu, S., & Herschman, H. R. (2019). A tumor agnostic therapeutic strategy for hexokinase 1–null/hexokinase 2–positive cancers. Cancer research, 79(23), 5907-5914.

Amendola, C. R., Mahaffey, J. P., Parker, S. J., Ahearn, I. M., Chen, W. C., Zhou, M., ... & Philips, M. R. (2019). KRAS4A directly regulates hexokinase 1. Nature, 576(7787), 482-486.

Xu, S., Catapang, A., Braas, D., Stiles, L., Doh, H. M., Lee, J. T., ... & Herschman, H. R. (2018). A precision therapeutic strategy for hexokinase 1-null, hexokinase 2-positive cancers. Cancer & metabolism, 6(1), 1-17.

Tseng, P. L., Chen, C. W., Hu, K. H., Cheng, H. C., Lin, Y. H., Tsai, W. H., ... & Chang, W. T. (2018). The decrease of glycolytic enzyme hexokinase 1 accelerates tumor malignancy via deregulating energy metabolism but sensitizes cancer cells to 2-deoxyglucose inhibition. Oncotarget, 9(27), 18949.

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

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