catalog number :
MBS9387438
products full name :
SDHA Antibody
products short name :
[SDHA]
other names :
[succinate dehydrogenase; Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial; succinate dehydrogenase complex flavoprotein subunit A; Flavoprotein subunit of complex II; Fp]
products gene name :
[SDHA]
other gene names :
[SDHA; SDHA; FP; PGL5; SDH1; SDH2; SDHF; CMD1GG; SDH2; SDHF; Fp]
reactivity :
Human, Mouse, Rat
specificity :
SDHA Ab detects endogenous levels of total SDHA
purity :
The antiserum was purified by peptide affinity chromatography using SulfoLink™ Coupling Resin (Thermo Fisher Scientific).
form :
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
storage stability :
Store at -20°C. Stable for 12 months from date of receipt.
tested application :
Western Blot (WB), Immunohistochemistry (IHC)
app notes :
WB 1:500-1:2000. IHC 1:50-1:200. IMPORTANT: For western blot, incubate membrane with diluted primary Abin 5% w/v milk , 1X TBS, 0.1% Tween®20 at 4°C with gentle shaking,overnight.
image1 heading :
Western Blot (WB)
other info1 :
Immunogen: A synthesized peptide derived from human SDHA. Subcellular Location: Mitochondrion inner membrane. Similarity: Belongs to the FAD-dependent oxidoreductase 2 family. FRD/SDH subfamily.
products description :
This gene encodes a major cata lytic subunit of succinateubiquinone oxidoreductase, a complex of the mitochondrial respiratory chain. The complex is composed of four nuclearencoded subunits and is localized in the mitochondrial inner membrane. Mutations in this gene have been associated with a form of mitochondrial respiratory chain deficiency known as Leigh Syndrome. A pseudogene has been identified on chromosome 3q29.
ncbi acc num :
NP_001281261.1
ncbi gb acc num :
NM_001294332.1
ncbi pathways :
Alzheimer's Disease Pathway (83097); Alzheimer's Disease Pathway (509); Carbon Metabolism Pathway (814926); Carbon Metabolism Pathway (817567); Citrate Cycle (TCA Cycle) Pathway (82927); Citrate Cycle (TCA Cycle) Pathway (288); Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway (855811); Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway (468202); Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate = Oxaloacetate Pathway (413348); Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate = Oxaloacetate Pathway (468204)
ncbi summary :
This gene encodes a major catalytic subunit of succinate-ubiquinone oxidoreductase, a complex of the mitochondrial respiratory chain. The complex is composed of four nuclear-encoded subunits and is localized in the mitochondrial inner membrane. Mutations in this gene have been associated with a form of mitochondrial respiratory chain deficiency known as Leigh Syndrome. A pseudogene has been identified on chromosome 3q29. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jun 2014]
uniprot summary :
SDHA: is the catalytic subunit of succinate dehydrogenase (SDH) complex II of the mitochondrial electron transport chain. Complex II contains four subunits: the flavoprotein catalytic subunit SDHA, iron-sulfur protein SDHB, and a cytochrome b560 composed of SDHC and SDHD. Interaction with SDH5 is required for FAD attachment. Responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). Defects in SDHA cause defective mitochondrial oxidative phosphorylation, giving rise to heterogeneous clinical symptoms ranging from isolated organ dysfunction to multisystem disorder. Acetylation of SDHA may control entry of the substrate into the active site, thus regulating its enzymatic activity. Acetylated SDHA may be a SIRT3 substrate SIRT3 is a mitochondrial NAD(+)-dependent deacetylase. Increased succinate levels as a consequence of SDH deficiency inhibit hypoxia inducible factor-1alpha (HIF-1alpha) prolyl hydroxylases leading to sustained HIF-1alpha expression in tumours. Defects in SDHA cause of Leigh syndrome, a severe disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. Protein type: Carbohydrate Metabolism - citrate (TCA) cycle; EC 1.3.5.1; Energy Metabolism - oxidative phosphorylation; Mitochondrial; Oxidoreductase; Tumor suppressor. Chromosomal Location of Human Ortholog: 5p15.33. Cellular Component: mitochondrial inner membrane; mitochondrial respiratory chain complex II; mitochondrion. Molecular Function: electron carrier activity; protein binding; succinate dehydrogenase (ubiquinone) activity; succinate dehydrogenase activity. Biological Process: anaerobic respiration; mitochondrial electron transport, succinate to ubiquinone; nervous system development; succinate metabolic process; tricarboxylic acid cycle. Disease: Cardiomyopathy, Dilated, 1gg; Leigh Syndrome; Mitochondrial Complex Ii Deficiency; Paragangliomas 5