Human COQ9 Protein

Description

Human COQ9 Protein
Catalog number: B2012010
Lot number: Batch Dependent
Expiration Date: Batch dependent
Amount: 100 µg
Molecular Weight or Concentration: 1 mg/mL
Supplied as: Solution
Applications: molecular tool for various biochemical applications
Storage: -20 °C
Keywords: ubiquinone biosynthesis protein COQ9 protein, mitochondrial protein, C16orf49 protein, COQ10D5 protein
Grade: Biotechnology grade. All products are highly pure. All solutions are made with Type I ultrapure water (resistivity >18 MΩ-cm) and are filtered through 0.22 um.

References:
1: García-Corzo L, Luna-Sánchez M, Doerrier C, García JA, Guarás A, Acín-Pérez R, Bullejos-Peregrín J, López A, Escames G, Enríquez JA, Acuña-Castroviejo D, López LC. Dysfunctional Coq9 protein causes predominant encephalomyopathy associated with CoQ deficiency Hum Mol Genet. 2013 Mar 15;22(6):1233-48.
2: Lohman DC, Forouhar F, Beebe ET, Stefely MS, Minogue CE, Ulbrich A, Stefely JA, Sukumar S, Luna-Sánchez M, Jochem A, Lew S, Seetharaman J, Xiao R, Wang H, Westphall MS, Wrobel RL, Everett JK, Mitchell JC, López LC, Coon JJ, Tong L, Pagliarini DJ. Mitochondrial COQ9 is a lipid-binding protein that associates with COQ7 to enable coenzyme Q biosynthesis Proc Natl Acad Sci U S A. 2014 Nov 4;111(44):E4697-705.
3: Li M, Thorne RF, Shi R, Zhang XD, Li J, Li J, Zhang Q, Wu M, Liu L. DDIT3 Directs a Dual Mechanism to Balance Glycolysis and Oxidative Phosphorylation during Glutamine Deprivation Adv Sci (Weinh). 2021 Jun;8(11):e2003732.
4: Yen HC, Liu YC, Kan CC, Wei HJ, Lee SH, Wei YH, Feng YH, Chen CW, Huang CC. Disruption of the human COQ5-containing protein complex is associated with diminished coenzyme Q10 levels under two different conditions of mitochondrial energy deficiency Biochim Biophys Acta. 2016 Sep;1860(9):1864-76.
5: Jin G, Kubo H, Kashiba M, Horinouchi R, Hasegawa M, Suzuki M, Sagawa T, Oizumi M, Fujisawa A, Tsukamoto H, Yoshimura S, Yamamoto Y. Saposin B is a human coenzyme q10-binding/transfer protein J Clin Biochem Nutr. 2008 Mar;42(2):167-74.
6: Kawamukai M. Biosynthesis of coenzyme Q in eukaryotes Biosci Biotechnol Biochem. 2016;80(1):23-33.
7: Luna-Sánchez M, Díaz-Casado E, Barca E, Tejada MÁ, Montilla-García Á, Cobos EJ, Escames G, Acuña-Castroviejo D, Quinzii CM, López LC. The clinical heterogeneity of coenzyme Q10 deficiency results from genotypic differences in the Coq9 gene EMBO Mol Med. 2015 May;7(5):670-87.
8: Smith AC, Ito Y, Ahmed A, Schwartzentruber JA, Beaulieu CL, Aberg E, Majewski J, Bulman DE, Horsting-Wethly K, Koning DV; Care4Rare Canada Consortium, Rodenburg RJ, Boycott KM, Penney LS. A family segregating lethal neonatal coenzyme Q(10) deficiency caused by mutations in COQ9 J Inherit Metab Dis. 2018 Jul;41(4):719-729.
9: Herebian D, Seibt A, Smits SHJ, Bünning G, Freyer C, Prokisch H, Karall D, Wredenberg A, Wedell A, López LC, Mayatepek E, Distelmaier F. Detection of 6-demethoxyubiquinone in CoQ(10) deficiency disorders: Insights into enzyme interactions and identification of potential therapeutics Mol Genet Metab. 2017 Jul;121(3):216-223.
10: Danhauser K, Herebian D, Haack TB, Rodenburg RJ, Strom TM, Meitinger T, Klee D, Mayatepek E, Prokisch H, Distelmaier F. Fatal neonatal encephalopathy and lactic acidosis caused by a homozygous loss-of-function variant in COQ9 Eur J Hum Genet. 2016 Mar;24(3):450-4.

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