Complex II subunit 70 kDa Fp monoclonal antibody
|
Catalog No. MS204
|
|
|
$355.00 - 100 µg
|
|
|
UniProt Number:
|
P31040
|
|
Alternate Names:
|
Succinate dehydrogenase [ubiquinone] flavoprotein subunit, Flavoprotein subunit of complex II, Fp, SDHA, SDH2, SDHF
|
|
Structure and Function:
|
Flavoprotein (Fp) is a subunit (664 AA) of the succinate dehydrogenase enzyme, which is a component of Complex II of the ETC. Succinate dehydrogenase transfers electrons from succinate to coenzyme Q (ubiquinone).
|
|
Disease Associations:
|
Abnormalities of the flavoprotein subunit are associated with complex II mitochondrial respiratory chain deficiency and succinate CoQ reductase deficiency, which can cause a wide range of pathological disorders affecting individual organs or entire body systems. In rare cases, a deficit in complex II can cause mitochondrial encephalomyopathy, leukodystrophy, late-onset optic atrophy and ataxia, myopathy with exercise intolerance, and isolated cardiomyopathy. Leigh syndrome is a pathology associated specifically with the flavoprotein subunit (SdhA) of succinate dehydrogenase. This syndrome presents itself with necrotic lesions that are located symmetrically in both sides the subcortical brain.
|
|
|
|
Product Specifications
|
|
|
Applications:
|
Western blotting, Immunocytochemistry (heat-induced antigen-retrieval improves signal), Immunohistochemistry, In-Cell ELISA, Blue Native Electrophoresis, Flow Cytometry
|
|
Species Reactivity:
|
human, bovine, rat, mouse, C. elegans
|
|
Host Species:
|
mouse
|
|
Isotype:
|
IgG1, κ
|
|
Clone ID:
|
2E3GC12FB2AE2
|
|
Concentration:
|
1 mg/mL in Hepes-Buffered Saline (HBS) with 0.02% azide as a preservative.
|
|
Suggested Working Concentration:
|
0.1 µg/mL for Western blotting
0.2 µg/mL for Immunocytochemistry
2 µg/mL for In-Cell ELISA (0.2 µg/well)
1 µg/mL for Flow Cytometry
|
|
Storage Conditions:
|
Store at 4°C. Do not freeze.
|
|
Country of Origin:
|
USA
|
Top
|
|
|
|
|
WB Images
(click to enlarge)
Figure 1. Isolated mitochondria from human heart (lane 1), bovine heart (lane 2), rat heart (lane 3), mouse heart (lane 4), and HepG2 (lane 5) detected with (MS204) anti-70kDa Fp antibody.
|
ICC Images
(click to enlarge)
Figure 2. Mitochondrial localization of complex II visualized by immunocytochemistry using anti-complex II subunit 70 kDa Fp mAb 2E3GC12FB2AE2 (MS204). Cultured human embryonic lung-derived fibroblasts (strain MRC5) were fixed, permeabilized and then labeled with MS204 (0.2 µg/ml) followed by an AlexaFluor® 488-conjugated-goat-anti-mouse IgG2a isotype specific secondary antibody (2 µg/ml).
|
IHC Images
(click to enlarge)
Figure 3. Skeletal muscle immunohistochemistry using MS204- fixed frozen tissue sections from a patient with a single large deletion of the mtDNA. All muscle fibers exhibit complex II immunoreactivity, consistent with the nuclear DNA-encoded expression pattern of this and all other subunits of complex II. Image kindly provided by Dr. J. Murphy and D. Turnbull, Mitochondrial Research Group, Newcastle University.
|
Flow Images
(click to enlarge)
Figure 4. HL-60 cells were stained with 1 µg/mL Complex II subunit 70kDa Fp antibody (MS204) (blue) or an equal amount of an isotype control antibody (red) and analyzed by flow cytometry.
|
Top
|
|
|
|
|
|
Published Studies Using This Product:
|
Ferreira et al., 2011. Progressive cavitating leukoencephalopathy associated with respiratory chain complex I deficiency and a novel mutation in NDUFS1.
Strub et al., 2010. Sphingosine-1-phosphate produced by sphingosine kinase 2 in mitochondria interacts with prohibitin 2 to regulate complex IV assembly and respiration.
Gerards et al., 2010. Riboflavin-responsive oxidative phosphorylation complex I deficiency caused by defective ACAD9: new function for an old gene.
Cameron et al., 2010. Complex V TMEM70 deficiency results in mitochondrial nucleoid disorganization.
Gegg et al., 2010. Mitofusin-1 and Mitofusin-2 are ubiquitinated in a PINK1/parkin dependent manner upon induction of mitophagy.
Fassone et al., 2010. FOXRED1, encoding a FAD-dependent oxidoreductase complex-I specific molecular chaperone, is mutated in infantile-onset mitochondrial encephalopathy.
Tuppen et al., 2010. The p.M292T NDUFS2 mutation causes complex I-deficient Leigh syndrome in multiple families.
Mayr et al., 2010. Mitochondrial ATP synthase deficiency due to a mutation in the ATP5E gene for the F1 {varepsilon} subunit.
Calore et al., 2010. Endosome-mitochondria juxtaposition during apoptosis induced by H. pylori VacA.
Ylikallio et al., 2010. High mitochondrial DNA copy number has detrimental effects in mice.
Walmsley et al., 2010. Proteomic analysis of brush-border membrane vesicles isolated from purified proximal convoluted tubules.
Ahola-Erkkila et al., 2010. Ketogenic diet slows down mitochondrial myopathy progression in mice.
Wydro et al., 2010. Targeting of the cytosolic poly(A) binding protein PABPC1 to mitochondria causes mitochondrial translation inhibition.
Cimen et al., 2010. Regulation of succinate dehydrogenase activity by SIRT3 in mammalian mitochondria.
Mracek et al., 2010. Downregulation of zinc-{alpha}2-glycoprotein in adipose tissue and liver of obese ob/ob mice and by tumour necrosis factor-alpha in adipocytes.
Feichtinger et al., 2010. Low aerobic mitochondrial energy metabolism in poorly- or undifferentiated neuroblastoma.
Fogal et al., 2010. Mitochondrial p32 Protein is a Critical Regulator of Tumor Metabolism via Maintenance of Oxidative Phosphorylation.
Cimen et al., 2010. Regulation of succinate dehydrogenase activity by SIRT3 in mammalian mitochondria.
Kachadourian et al., 2009. Casiopeína IIgly-induced oxidative stress and mitochondrial dysfunction in human lung cancer A549 and H157 cells.
Horvath et al., 2009. Molecular basis of infantile reversible cytochrome c oxidase deficiency myopathy.
Gómez et al., 2009. Supercomplexes of the mitochondrial electron transport chain decline in the aging rat heart.
Caro et al., 2009. Effect of 40% restriction of dietary amino acids (except methionine) on mitochondrial oxidative stress and biogenesis, AIF and SIRT1 in rat liver.
Gerards et al., 2009. Defective complex I assembly due to C20orf7 mutations as a new cause of Leigh syndrome.
Brennan et al., 2009. Deletion of mouse MsrA results in HBO-induced cataract: MsrA repairs mitochondrial cytochrome c.
De Maria et al., 2009. Calpain expression and activity during lens fiber cell differentiation.
Yahata et al., 2009. Nicotinamide mononucleotide adenylyltransferase expression in mitochondrial matrix delays Wallerian degeneration.
Jeong et al., 2009. Modulation of the caveolin-3 localization to caveolae and STAT3 to mitochondria by catecholamine-induced cardiac hypertrophy in H9c2 cardiomyoblasts.
Hanein et al., 2009. TMEM126A, encoding a mitochondrial protein, is mutated in autosomal-recessive nonsyndromic optic atrophy.
Son et al., 2009. Redox susceptibility of SOD1 mutants is associated with the differential response to CCS over-expression in vivo.
Murayama et al., 2009. Intractable secretory diarrhea in a Japanese boy with mitochondrial respiratory chain complex I deficiency.
Hakonen et al., 2008. Infantile-onset spinocerebellar ataxia and mitochondrial recessive ataxia syndrome are associated with neuronal complex I defect and mtDNA depletion.
Calvaruso et al., 2008. Electrophoresis techniques to investigate defects in oxidative phosphorylation.
Rorbach et al., 2008. The human mitochondrial ribosome recycling factor is essential for cell viability.
Ahn et al., 2008. A role for the mitochondrial deacetylase Sirt3 in regulating energy homeostasis.
Lyly et al., 2008. Deficiency of the INCL protein Ppt1 results in changes in ectopic F1-ATP synthase and altered cholesterol metabolism.
Son et al., 2008. Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein.
Fornuskova et al., 2008. The impact of mitochondrial tRNA mutations on the amount of ATP synthase differs in the brain compared to other tissues.
Lebon et al., 2007. A novel mutation of the NDUFS7 gene leads to activation of a cryptic exon and impaired assembly of mitochondrial complex I in a patient with Leigh syndrome.
Canal et al., 2007. Regulation of the cysteine desulfurase Nfs1 and the scaffold protein IscU in macrophages stimulated with interferon-gamma and lipopolysaccharide.
Ramanujan et al., 2007. Aging process modulates nonlinear dynamics in liver cell metabolism.
Li et al., 2007. An assembled complex IV maintains the stability and activity of complex I in mammalian mitochondria.
Park et al., 2007. Yeast NDI1 improves oxidative phosphorylation capacity and increases protection against oxidative stress and cell death in cells carrying a Leber's hereditary optic neuropathy mutation.
Folbergrová et al., 2007. Mitochondrial complex I inhibition in cerebral cortex of immature rats following homocysteic acid-induced seizures.
Carrozzo et al., 2007. SUCLA2 mutations are associated with mild methylmalonic aciduria, Leigh-like encephalomyopathy, dystonia and deafness.
Yoon et al., 2006. Formation of elongated giant mitochondria in DFO-induced cellular senescence: involvement of enhanced fusion process through modulation of Fis1.
Wang et al., 2006. Human mitochondrial DNA nucleoids are linked to protein folding machinery and metabolic enzymes at the mitochondrial inner membrane.
Honzík et al., 2006. Specific properties of heavy fraction of mitochondria from human-term placenta - glycerophosphate-dependent hydrogen peroxide production.
Flachs et al., 2005. Polyunsaturated fatty acids of marine origin upregulate mitochondrial biogenesis and induce beta-oxidation in white fat.
Ogilvie et al., 2005. A molecular chaperone for mitochondrial complex I assembly is mutated in a progressive encephalopathy.
Jesina et al., 2004. Diminished synthesis of subunit a (ATP6) and altered function of ATP synthase and cytochrome c oxidase due to the mtDNA 2 bp microdeletion of TA at positions 9205 and 9206.
Yin et al., 2004. Alteration of the copy number and deletion of mitochondrial DNA in human hepatocellular carcinoma.
|
Top
|
|
|
|
|
CART
