SDHC blocking peptide
SDHC Blocking Peptide (C-Term)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes one of four nuclear-encoded subunits that comprise succinate dehydrogenase, also known as mitochondrial complex II, a key enzyme complex of the tricarboxylic acid cycle and aerobic respiratory chains of mitochondria. The encoded protein is one of two integral membrane proteins that anchor other subunits of the complex, which form the catalytic core, to the inner mitochondrial membrane. There are several related pseudogenes for this gene on different chromosomes. Mutations in this gene have been associated with paragangliomas. Alternatively spliced transcript variants have been described. [provided by RefSeq, May 2013]
Uniprot Description
SDHC: Membrane-anchoring 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). Defects in SDHC are the cause of paragangliomas type 3 (PGL3). A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion. Paragangliomas are most commonly located in the head and neck region, specifically at the carotid bifurcation, the jugular foramen, the vagal nerve, and in the middle ear. Defects in SDHC are a cause of paraganglioma and gastric stromal sarcoma (PGGSS); also known as Carney- Stratakis syndrome. 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. Belongs to the cytochrome b560 family. 5 isoforms of the human protein are produced by alternative splicing.
Protein type: Mitochondrial; Membrane protein, integral; Membrane protein, multi-pass; Energy Metabolism - oxidative phosphorylation; Carbohydrate Metabolism - citrate (TCA) cycle
Chromosomal Location of Human Ortholog: 1q23.3
Cellular Component: integral to membrane; mitochondrial inner membrane; mitochondrial respiratory chain complex II; mitochondrion
Molecular Function: heme binding
Biological Process: aerobic respiration; mitochondrial electron transport, succinate to ubiquinone; tricarboxylic acid cycle
Disease: Gastrointestinal Stromal Tumor; Paraganglioma And Gastric Stromal Sarcoma; Paragangliomas 3
Research Articles on SDHC
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Product Notes
The SDHC sdhc (Catalog #AAA9230467) is a Blocking Peptide and is intended for research purposes only. The product is available for immediate purchase. It is sometimes possible for the material contained within the vial of "SDHC, Blocking Peptide" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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