Mouse Anti-SDHA Recombinant Antibody (CBXS-4539) (CBMAB-S1779-CQ)

Succinate Dehydrogenase Complex Flavoprotein Subunit A

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.

Succinate Dehydrogenase Complex Flavoprotein Subunit A; Flavoprotein Subunit Of Complex II; Succinate Dehydrogenase Complex, Subunit A, Flavoprotein (Fp); SDH2; SDHF; FP; Succinate Dehydrogenase [Ubiquinone] Flavoprotein Subunit, Mitochondrial;

Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (PubMed:24781757).
Can act as a tumor suppressor (PubMed:20484225).

Biological Process mitochondrial electron transport, succinate to ubiquinoneManual Assertion Based On ExperimentIBA:GO_Central
Biological Process nervous system developmentManual Assertion Based On ExperimentIMP:UniProtKB
Biological Process proton motive force-driven mitochondrial ATP synthesis1 PublicationIC:ComplexPortal
Biological Process respiratory electron transport chainManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process succinate metabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process tricarboxylic acid cycleManual Assertion Based On ExperimentTAS:UniProtKB

Mitochondrion inner membrane

Mitochondrial complex II deficiency, nuclear type 1 (MC2DN1):
A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations. Clinical features include psychomotor regression in infants, poor growth with lack of speech development, severe spastic quadriplegia, dystonia, progressive leukoencephalopathy, muscle weakness, exercise intolerance, cardiomyopathy. Some patients manifest Leigh syndrome or Kearns-Sayre syndrome. MC2DN1 inheritance is autosomal recessive.
Leigh syndrome (LS):
An early-onset progressive neurodegenerative disorder characterized by the presence of focal, bilateral lesions in one or more areas of the central nervous system including the brainstem, thalamus, basal ganglia, cerebellum and spinal cord. Clinical features depend on which areas of the central nervous system are involved and include subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, and dysphagia.
Cardiomyopathy, dilated 1GG (CMD1GG):
A disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
Paragangliomas 5 (PGL5):
A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion. Paragangliomas can develop at various body sites, including the head, neck, thorax and abdomen. Most commonly, they are located in the head and neck region, specifically at the carotid bifurcation, the jugular foramen, the vagal nerve, and in the middle ear.
Neurodegeneration with ataxia and late-onset optic atrophy (NDAXOA):
An autosomal dominant disorder characterized by slowly progressive cerebellar and gait ataxia, optic atrophy, and myopathy or myalgia. Additional features can include cardiomyopathy, psychiatric disturbances, and peripheral sensory impairment. Disease onset is usually in mid-adulthood.

Phosphorylation at Tyr-215 is important for efficient electron transfer in complex II and the prevention of ROS generation.
Acetylated. Deacetylated by SIRT3.