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- References [22]
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- Western blot [1]
- Immunohistochemistry [2]
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- Product number
- 15083-1-AP - Provider product page
- Provider
- Proteintech Group
- Proper citation
- Proteintech Cat#15083-1-AP, RRID:AB_2082207
- Product name
- COQ7 antibody
- Antibody type
- Polyclonal
- Description
- KD/KO validated COQ7 antibody (Cat. #15083-1-AP) is a rabbit polyclonal antibody that shows reactivity with human, mouse, rat and has been validated for the following applications: IHC, IP, WB, ELISA.
- Reactivity
- Human, Mouse, Rat
- Host
- Rabbit
- Conjugate
- Unconjugated
- Isotype
- IgG
- Vial size
- 20ul, 150ul
Submitted references 4-Hydroxybenzoic acid rescues multisystemic disease and perinatal lethality in a mouse model of mitochondrial disease.
Homozygous variant in COQ7 causes autosomal recessive hereditary spastic paraplegia.
Novel Homozygous Variant in COQ7 in Siblings With Hereditary Motor Neuropathy.
Homozygous COQ7 mutation: a new cause of potentially treatable distal hereditary motor neuropathy.
The CoQ biosynthetic di-iron carboxylate hydroxylase COQ7 is inhibited by in vivo metalation with manganese but remains functional by metalation with cobalt.
The complex formation of MASP-3 with pattern recognition molecules of the lectin complement pathway retains MASP-3 in the circulation.
Human COQ4 deficiency: delineating the clinical, metabolic and neuroimaging phenotypes.
The Q-junction and the inflammatory response are critical pathological and therapeutic factors in CoQ deficiency.
A novel COQ7 mutation causing primarily neuromuscular pathology and its treatment options.
Dysregulation of iron homeostasis and methamphetamine reward behaviors in Clk1-deficient mice.
Cold-inducible RNA binding protein agonist enhances the cardioprotective effect of UW solution during extended heart preservation.
Resveratrol Regulates the Expression of Genes Involved in CoQ Synthesis in Liver in Mice Fed with High Fat Diet.
Hepatic HuR modulates lipid homeostasis in response to high-fat diet.
β-RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9(R239X) mice.
Reduction in the levels of CoQ biosynthetic proteins is related to an increase in lifespan without evidence of hepatic mitohormesis.
Detection of 6-demethoxyubiquinone in CoQ(10) deficiency disorders: Insights into enzyme interactions and identification of potential therapeutics.
Activation of AMPK/mTORC1-Mediated Autophagy by Metformin Reverses Clk1 Deficiency-Sensitized Dopaminergic Neuronal Death.
4-Hydroxybenzoic acid restores CoQ(10) biosynthesis in human COQ2 deficiency.
Clk1-regulated aerobic glycolysis is involved in glioma chemoresistance.
Fatal neonatal encephalopathy and lactic acidosis caused by a homozygous loss-of-function variant in COQ9.
A nuclear role for the respiratory enzyme CLK-1 in regulating mitochondrial stress responses and longevity.
ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption.
Corral-Sarasa J, Martínez-Gálvez JM, González-García P, Wendling O, Jiménez-Sánchez L, López-Herrador S, Quinzii CM, Díaz-Casado ME, López LC
Cell reports 2024 May 28;43(5):114148
Cell reports 2024 May 28;43(5):114148
Homozygous variant in COQ7 causes autosomal recessive hereditary spastic paraplegia.
Qiu Y, Xiong Y, Wang L, Zhu M, Tan D, Hong D
Annals of clinical and translational neurology 2024 Apr;11(4):1067-1074
Annals of clinical and translational neurology 2024 Apr;11(4):1067-1074
Novel Homozygous Variant in COQ7 in Siblings With Hereditary Motor Neuropathy.
Smith IC, Pileggi CA, Wang Y, Kernohan K, Hartley T, McMillan HJ, Sampaio ML, Melkus G, Woulfe J, Parmar G, Bourque PR, Breiner A, Zwicker J, Pringle CE, Jarinova O, Lochmüller H, Dyment DA, Brais B, Boycott KM, Care4Rare Canada Consortium,, Hekimi S, Harper ME, Warman-Chardon J
Neurology. Genetics 2023 Feb;9(1):e200048
Neurology. Genetics 2023 Feb;9(1):e200048
Homozygous COQ7 mutation: a new cause of potentially treatable distal hereditary motor neuropathy.
Jacquier A, Theuriet J, Fontaine F, Mosbach V, Lacoste N, Ribault S, Risson V, Carras J, Coudert L, Simonet T, Latour P, Stojkovic T, Piard J, Cosson A, Lesca G, Bouhour F, Allouche S, Puccio H, Pegat A, Schaeffer L
Brain : a journal of neurology 2023 Aug 1;146(8):3470-3483
Brain : a journal of neurology 2023 Aug 1;146(8):3470-3483
The CoQ biosynthetic di-iron carboxylate hydroxylase COQ7 is inhibited by in vivo metalation with manganese but remains functional by metalation with cobalt.
Wang Y, Hekimi S
microPublication biology 2022;2022
microPublication biology 2022;2022
The complex formation of MASP-3 with pattern recognition molecules of the lectin complement pathway retains MASP-3 in the circulation.
Kusakari K, Machida T, Ishida Y, Omori T, Suzuki T, Sekimata M, Wada I, Fujita T, Sekine H
Frontiers in immunology 2022;13:907023
Frontiers in immunology 2022;13:907023
Human COQ4 deficiency: delineating the clinical, metabolic and neuroimaging phenotypes.
Laugwitz L, Seibt A, Herebian D, Peralta S, Kienzle I, Buchert R, Falb R, Gauck D, Müller A, Grimmel M, Beck-Woedel S, Kern J, Daliri K, Katibeh P, Danhauser K, Leiz S, Alesi V, Baertling F, Vasco G, Steinfeld R, Wagner M, Caglayan AO, Gumus H, Burmeister M, Mayatepek E, Martinelli D, Tamhankar PM, Tamhankar V, Joset P, Steindl K, Rauch A, Bonnen PE, Froukh T, Groeschel S, Krägeloh-Mann I, Haack TB, Distelmaier F
Journal of medical genetics 2022 Sep;59(9):878-887
Journal of medical genetics 2022 Sep;59(9):878-887
The Q-junction and the inflammatory response are critical pathological and therapeutic factors in CoQ deficiency.
González-García P, Díaz-Casado ME, Hidalgo-Gutiérrez A, Jiménez-Sánchez L, Bakkali M, Barriocanal-Casado E, Escames G, Chiozzi RZ, Völlmy F, Zaal EA, Berkers CR, Heck AJR, López LC
Redox biology 2022 Sep;55:102403
Redox biology 2022 Sep;55:102403
A novel COQ7 mutation causing primarily neuromuscular pathology and its treatment options.
Wang Y, Gumus E, Hekimi S
Molecular genetics and metabolism reports 2022 Jun;31:100877
Molecular genetics and metabolism reports 2022 Jun;31:100877
Dysregulation of iron homeostasis and methamphetamine reward behaviors in Clk1-deficient mice.
Yan PJ, Ren ZX, Shi ZF, Wan CL, Han CJ, Zhu LS, Li NN, Waddington JL, Zhen XC
Acta pharmacologica Sinica 2022 Jul;43(7):1686-1698
Acta pharmacologica Sinica 2022 Jul;43(7):1686-1698
Cold-inducible RNA binding protein agonist enhances the cardioprotective effect of UW solution during extended heart preservation.
Pan HZ, Zhang LJ, Liu YW, Li YN, Su ZH, Meng J, Zhang H
Artificial organs 2020 Oct;44(10):E406-E418
Artificial organs 2020 Oct;44(10):E406-E418
Resveratrol Regulates the Expression of Genes Involved in CoQ Synthesis in Liver in Mice Fed with High Fat Diet.
Meza-Torres C, Hernández-Camacho JD, Cortés-Rodríguez AB, Fang L, Bui Thanh T, Rodríguez-Bies E, Navas P, López-Lluch G
Antioxidants (Basel, Switzerland) 2020 May 15;9(5)
Antioxidants (Basel, Switzerland) 2020 May 15;9(5)
Hepatic HuR modulates lipid homeostasis in response to high-fat diet.
Zhang Z, Zong C, Jiang M, Hu H, Cheng X, Ni J, Yi X, Jiang B, Tian F, Chang MW, Su W, Zhu L, Li J, Xiang X, Miao C, Gorospe M, de Cabo R, Dou Y, Ju Z, Yang J, Jiang C, Yang Z, Wang W
Nature communications 2020 Jun 16;11(1):3067
Nature communications 2020 Jun 16;11(1):3067
β-RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9(R239X) mice.
Hidalgo-Gutiérrez A, Barriocanal-Casado E, Bakkali M, Díaz-Casado ME, Sánchez-Maldonado L, Romero M, Sayed RK, Prehn C, Escames G, Duarte J, Acuña-Castroviejo D, López LC
EMBO molecular medicine 2019 Jan;11(1)
EMBO molecular medicine 2019 Jan;11(1)
Reduction in the levels of CoQ biosynthetic proteins is related to an increase in lifespan without evidence of hepatic mitohormesis.
Rodríguez-Hidalgo M, Luna-Sánchez M, Hidalgo-Gutiérrez A, Barriocanal-Casado E, Mascaraque C, Acuña-Castroviejo D, Rivera M, Escames G, López LC
Scientific reports 2018 Sep 18;8(1):14013
Scientific reports 2018 Sep 18;8(1):14013
Detection of 6-demethoxyubiquinone in CoQ(10) deficiency disorders: Insights into enzyme interactions and identification of potential therapeutics.
Herebian D, Seibt A, Smits SHJ, Bünning G, Freyer C, Prokisch H, Karall D, Wredenberg A, Wedell A, López LC, Mayatepek E, Distelmaier F
Molecular genetics and metabolism 2017 Jul;121(3):216-223
Molecular genetics and metabolism 2017 Jul;121(3):216-223
Activation of AMPK/mTORC1-Mediated Autophagy by Metformin Reverses Clk1 Deficiency-Sensitized Dopaminergic Neuronal Death.
Yan Q, Han C, Wang G, Waddington JL, Zheng L, Zhen X
Molecular pharmacology 2017 Dec;92(6):640-652
Molecular pharmacology 2017 Dec;92(6):640-652
4-Hydroxybenzoic acid restores CoQ(10) biosynthesis in human COQ2 deficiency.
Herebian D, Seibt A, Smits SHJ, Rodenburg RJ, Mayatepek E, Distelmaier F
Annals of clinical and translational neurology 2017 Dec;4(12):902-908
Annals of clinical and translational neurology 2017 Dec;4(12):902-908
Clk1-regulated aerobic glycolysis is involved in glioma chemoresistance.
Zhang L, Yang H, Zhang W, Liang Z, Huang Q, Xu G, Zhen X, Zheng LT
Journal of neurochemistry 2017 Aug;142(4):574-588
Journal of neurochemistry 2017 Aug;142(4):574-588
Fatal neonatal encephalopathy and lactic acidosis caused by a homozygous loss-of-function variant in COQ9.
Danhauser K, Herebian D, Haack TB, Rodenburg RJ, Strom TM, Meitinger T, Klee D, Mayatepek E, Prokisch H, Distelmaier F
European journal of human genetics : EJHG 2016 Mar;24(3):450-4
European journal of human genetics : EJHG 2016 Mar;24(3):450-4
A nuclear role for the respiratory enzyme CLK-1 in regulating mitochondrial stress responses and longevity.
Monaghan RM, Barnes RG, Fisher K, Andreou T, Rooney N, Poulin GB, Whitmarsh AJ
Nature cell biology 2015 Jun;17(6):782-92
Nature cell biology 2015 Jun;17(6):782-92
ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption.
Ashraf S, Gee HY, Woerner S, Xie LX, Vega-Warner V, Lovric S, Fang H, Song X, Cattran DC, Avila-Casado C, Paterson AD, Nitschké P, Bole-Feysot C, Cochat P, Esteve-Rudd J, Haberberger B, Allen SJ, Zhou W, Airik R, Otto EA, Barua M, Al-Hamed MH, Kari JA, Evans J, Bierzynska A, Saleem MA, Böckenhauer D, Kleta R, El Desoky S, Hacihamdioglu DO, Gok F, Washburn J, Wiggins RC, Choi M, Lifton RP, Levy S, Han Z, Salviati L, Prokisch H, Williams DS, Pollak M, Clarke CF, Pei Y, Antignac C, Hildebrandt F
The Journal of clinical investigation 2013 Dec;123(12):5179-89
The Journal of clinical investigation 2013 Dec;123(12):5179-89
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Supportive validation
- Submitted by
- Proteintech Group (provider)
- Main image
- Experimental details
- mouse liver tissue were subjected to SDS PAGE followed by western blot with 15083-1-AP(COQ7 antibody) at dilution of 1:500
- Sample type
- tissue
Supportive validation
- Submitted by
- Proteintech Group (provider)
- Main image
- Experimental details
- Immunohistochemical of paraffin-embedded human liver cancer using 15083-1-AP(COQ7 antibody) at dilution of 1:100 (under 10x lens)
- Sample type
- tissue
- Submitted by
- Proteintech Group (provider)
- Main image
- Experimental details
- The COQ7 antibody from Proteintech is a rabbit polyclonal antibody to a recombinant protein of human COQ7. This antibody recognizes human,mouse,rat antigen. The COQ7 antibody has been validated for the following applications: ELISA, WB, IHC analysis.
