SDHB
GeneName
SDHB
Summary
SDHB, also known as SDH or SDH1, is a 32 kDa enzyme that is a crucial component of the succinate dehydrogenase complex, which is located in the mitochondrial inner membrane. This enzyme plays a pivotal role in the tricarboxylic acid cycle and is involved in the mitochondrial electron transport chain, facilitating the conversion of succinate to fumarate while transferring electrons to ubiquinone. SDHB is essential for aerobic respiration and is implicated in the generation of the proton motive force that drives ATP synthesis. It is expressed in various tissues, particularly those with high energy demands, such as cardiac and skeletal muscle, and is associated with the mitochondrial matrix and respiratory chain complex II.
Importance
SDHB is relevant to: - Cancer research, particularly in the context of hereditary paraganglioma and pheochromocytoma due to its role in the regulation of cellular respiration and energy metabolism - Mitochondrial dysfunction studies, as mutations in SDHB can lead to impaired oxidative phosphorylation and contribute to various metabolic disorders - Understanding the mechanisms of reactive oxygen species production in mitochondria, which is linked to aging and age-related diseases - Investigating the interplay between metabolism and signalling pathways, especially in the context of hypoxia and tumour microenvironments
Top Products
For researchers investigating SDHB, we recommend two excellent primary antibodies. The first is the well-cited Anti-SDHB antibody [21A11AE7] (ab14714), which has garnered 304 citations, reflecting its strong reputation in the field. This monoclonal antibody is validated for use in Western blotting (WB), immunohistochemistry (IHC), immunocytochemistry (ICC), and flow cytometry (FC), making it a versatile choice for various applications. Importantly, it has also been validated in knockout models, ensuring reliable results in your experiments.Additionally, we offer the recombinant Anti-SDHB antibody [EPR13042(B)] (ab178423), which is also validated in knockout models and suitable for WB, IHC, immunoprecipitation (IP), and FC. With 24 citations, this recombinant antibody provides the batch-to-batch consistency that researchers often seek. Together, these antibodies provide robust options for studying SDHB effectively.
Abcam Product Citation Summary
The data indicates that SDHB is frequently studied in the context of mitochondrial function and related diseases, particularly in human cells and tissues. The use of various Abcam antibodies in Western blotting highlights the importance of SDHB in mitochondrial respiration, lipotoxicity, and cancer research, among other areas. The studies span a range of species, including human, mouse, and rat, suggesting a broad interest in understanding the role of SDHB across different biological systems.
Abcam Product Citation Table
Function
Iron-sulfur protein (IP) subunit of the succinate dehydrogenase complex (mitochondrial respiratory chain complex II), responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (PubMed:26925370, PubMed:27604842). SDH also oxidizes malate to the non-canonical enol form of oxaloacetate, enol-oxaloacetate (By similarity). Enol-oxaloacetate, which is a potent inhibitor of the succinate dehydrogenase activity, is further isomerized into keto-oxaloacetate (By similarity).
Involvement in disease
Pheochromocytoma/paraganglioma syndrome 4
PPGL4
A form of pheochromocytoma/paraganglioma syndrome, a tumor predisposition syndrome characterized by the development of neuroendocrine tumors, usually in adulthood. Pheochromocytomas are catecholamine-producing tumors that arise from chromaffin cells in the adrenal medulla. Paragangliomas develop from sympathetic paraganglia in the thorax, abdomen, and pelvis, as well as from parasympathetic paraganglia in the head and neck. PPGL4 inheritance is autosomal dominant.
None
The disease is caused by variants affecting the gene represented in this entry.
Paraganglioma and gastric stromal sarcoma
PGGSS
Gastrointestinal stromal tumors may be sporadic or inherited in an autosomal dominant manner, alone or as a component of a syndrome associated with other tumors, such as in the context of neurofibromatosis type 1 (NF1). Patients have both gastrointestinal stromal tumors and paragangliomas. Susceptibility to the tumors was inherited in an apparently autosomal dominant manner, with incomplete penetrance.
None
The disease is caused by variants affecting the gene represented in this entry.
Mitochondrial complex II deficiency, nuclear type 4
MC2DN4
A form of mitochondrial complex II deficiency, a disorder with heterogeneous clinical manifestations. Some patients have multisystem involvement of the brain, heart, muscle, liver, and kidneys resulting in death in infancy, whereas others have only isolated cardiac or muscle involvement with onset in adulthood and normal cognition. 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. MC2DN4 is a severe, autosomal recessive form characterized by early-onset progressive neurodegeneration with leukoencephalopathy.
None
The disease is caused by variants affecting the gene represented in this entry.
Pathway
Carbohydrate metabolism; tricarboxylic acid cycle; fumarate from succinate (eukaryal route): step 1/1.
Sequence Similarities
Belongs to the succinate dehydrogenase/fumarate reductase iron-sulfur protein family.
Cellular localization
- Mitochondrion inner membrane
- Peripheral membrane protein
- Matrix side
Alternative names
SDH, SDH1, SDHB, Iron-sulfur subunit of complex II, Malate dehydrogenase [quinone] iron-sulfur subunit, Ip