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- Antigen structure
- References [10]
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- Validations
- Western blot [1]
- Immunohistochemistry [2]
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- Product number
- 16720-1-AP - Provider product page
- Provider
- Proteintech Group
- Proper citation
- Proteintech Cat#16720-1-AP, RRID:AB_2298378
- Product name
- NDUFB11 antibody
- Antibody type
- Polyclonal
- Description
- KD/KO validated NDUFB11 antibody (Cat. #16720-1-AP) is a rabbit polyclonal antibody that shows reactivity with human, mouse, rat and has been validated for the following applications: IF, IHC, IP, WB, ELISA.
- Reactivity
- Human, Mouse, Rat
- Host
- Rabbit
- Conjugate
- Unconjugated
- Isotype
- IgG
- Vial size
- 20ul, 150ul
Submitted references Identification of TMEM126A as OXA1L-interacting protein reveals cotranslational quality control in mitochondria.
Mutational burden of XPNPEP3 leads to defects in mitochondrial complex I and cilia in NPHPL1.
NDUFS3 depletion permits complex I maturation and reveals TMEM126A/OPA7 as an assembly factor binding the ND4-module intermediate.
Complex I mutations synergize to worsen the phenotypic expression of Leber's hereditary optic neuropathy.
Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV.
miR-181a/b downregulation exerts a protective action on mitochondrial disease models.
A novel mutation in NDUFB11 unveils a new clinical phenotype associated with lactic acidosis and sideroblastic anemia.
DJ-1 modulates mitochondrial response to oxidative stress: clues from a novel diagnosis of PARK7.
Novel mutation in mitochondrial Elongation Factor EF-Tu associated to dysplastic leukoencephalopathy and defective mitochondrial DNA translation.
Mutations in NDUFB11, encoding a complex I component of the mitochondrial respiratory chain, cause microphthalmia with linear skin defects syndrome.
Poerschke S, Oeljeklaus S, Cruz-Zaragoza LD, Schenzielorz A, Dahal D, Hillen HS, Das H, Kremer LS, Valpadashi A, Breuer M, Sattmann J, Richter-Dennerlein R, Warscheid B, Dennerlein S, Rehling P
Molecular cell 2024 Jan 18;84(2):345-358.e5
Molecular cell 2024 Jan 18;84(2):345-358.e5
Mutational burden of XPNPEP3 leads to defects in mitochondrial complex I and cilia in NPHPL1.
Tong L, Rao J, Yang C, Xu J, Lu Y, Zhang Y, Cang X, Xie S, Mao J, Jiang P
iScience 2023 Aug 18;26(8):107446
iScience 2023 Aug 18;26(8):107446
NDUFS3 depletion permits complex I maturation and reveals TMEM126A/OPA7 as an assembly factor binding the ND4-module intermediate.
D'Angelo L, Astro E, De Luise M, Kurelac I, Umesh-Ganesh N, Ding S, Fearnley IM, Gasparre G, Zeviani M, Porcelli AM, Fernandez-Vizarra E, Iommarini L
Cell reports 2021 Apr 20;35(3):109002
Cell reports 2021 Apr 20;35(3):109002
Complex I mutations synergize to worsen the phenotypic expression of Leber's hereditary optic neuropathy.
Ji Y, Zhang J, Lu Y, Yi Q, Chen M, Xie S, Mao X, Xiao Y, Meng F, Zhang M, Yang R, Guan MX
The Journal of biological chemistry 2020 Sep 18;295(38):13224-13238
The Journal of biological chemistry 2020 Sep 18;295(38):13224-13238
Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV.
Protasoni M, Pérez-Pérez R, Lobo-Jarne T, Harbour ME, Ding S, Peñas A, Diaz F, Moraes CT, Fearnley IM, Zeviani M, Ugalde C, Fernández-Vizarra E
The EMBO journal 2020 Feb 3;39(3):e102817
The EMBO journal 2020 Feb 3;39(3):e102817
miR-181a/b downregulation exerts a protective action on mitochondrial disease models.
Indrieri A, Carrella S, Romano A, Spaziano A, Marrocco E, Fernandez-Vizarra E, Barbato S, Pizzo M, Ezhova Y, Golia FM, Ciampi L, Tammaro R, Henao-Mejia J, Williams A, Flavell RA, De Leonibus E, Zeviani M, Surace EM, Banfi S, Franco B
EMBO molecular medicine 2019 May;11(5)
EMBO molecular medicine 2019 May;11(5)
A novel mutation in NDUFB11 unveils a new clinical phenotype associated with lactic acidosis and sideroblastic anemia.
Torraco A, Bianchi M, Verrigni D, Gelmetti V, Riley L, Niceta M, Martinelli D, Montanari A, Guo Y, Rizza T, Diodato D, Di Nottia M, Lucarelli B, Sorrentino F, Piemonte F, Francisci S, Tartaglia M, Valente EM, Dionisi-Vici C, Christodoulou J, Bertini E, Carrozzo R
Clinical genetics 2017 Mar;91(3):441-447
Clinical genetics 2017 Mar;91(3):441-447
DJ-1 modulates mitochondrial response to oxidative stress: clues from a novel diagnosis of PARK7.
Di Nottia M, Masciullo M, Verrigni D, Petrillo S, Modoni A, Rizzo V, Di Giuda D, Rizza T, Niceta M, Torraco A, Bianchi M, Santoro M, Bentivoglio AR, Bertini E, Piemonte F, Carrozzo R, Silvestri G
Clinical genetics 2017 Jul;92(1):18-25
Clinical genetics 2017 Jul;92(1):18-25
Novel mutation in mitochondrial Elongation Factor EF-Tu associated to dysplastic leukoencephalopathy and defective mitochondrial DNA translation.
Di Nottia M, Montanari A, Verrigni D, Oliva R, Torraco A, Fernandez-Vizarra E, Diodato D, Rizza T, Bianchi M, Catteruccia M, Zeviani M, Dionisi-Vici C, Francisci S, Bertini E, Carrozzo R
Biochimica et biophysica acta. Molecular basis of disease 2017 Apr;1863(4):961-967
Biochimica et biophysica acta. Molecular basis of disease 2017 Apr;1863(4):961-967
Mutations in NDUFB11, encoding a complex I component of the mitochondrial respiratory chain, cause microphthalmia with linear skin defects syndrome.
van Rahden VA, Fernandez-Vizarra E, Alawi M, Brand K, Fellmann F, Horn D, Zeviani M, Kutsche K
American journal of human genetics 2015 Apr 2;96(4):640-50
American journal of human genetics 2015 Apr 2;96(4):640-50
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Supportive validation
- Submitted by
- Proteintech Group (provider)
- Main image
- Experimental details
- HepG2 cells were subjected to SDS PAGE followed by western blot with 16720-1-AP(NDUFB11 antibody) at dilution of 1:800
- Sample type
- cell line
Supportive validation
- Submitted by
- Proteintech Group (provider)
- Main image
- Experimental details
- Immunohistochemical of paraffin-embedded human kidney using 16720-1-AP(NDUFB11 antibody) at dilution of 1:100 (under 10x lens)
- Sample type
- tissue
- Submitted by
- Proteintech Group (provider)
- Main image
- Experimental details
- The NDUFB11 antibody from Proteintech is a rabbit polyclonal antibody to a recombinant protein of human NDUFB11. This antibody recognizes human, mouse, rat antigen. The NDUFB11 antibody has been validated for the following applications: ELISA, WB, IHC analysis.
