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The succinate dehydrogenase (SDH) complex (or complex II) of the mitochondrial respiratory chain is composed of 4 individual subunits. Additionally we are shipping SDHAF1 Proteins (5) and SDHAF1 Kits (4) and many more products for this protein.
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Dog (Canine) Polyclonal SDHAF1 Primary Antibody for EIA, WB - ABIN452676
Ghezzi, Goffrini, Uziel, Horvath, Klopstock, Lochmüller, DAdamo, Gasparini, Strom, Prokisch, Invernizzi, Ferrero, Zeviani: SDHAF1, encoding a LYR complex-II specific assembly factor, is mutated in SDH-defective infantile leukoencephalopathy. in Nature genetics 2010
that SDHAF1 contributes to iron-sulfur (Fe-S) cluster incorporation into the Fe-S subunit of CII, SDHB (show SDHB Antibodies).
Studies indicate that an array of tumor syndromes caused by complex II-associated mutations in genes SDHA (show SDHA Antibodies), SDHB (show SDHB Antibodies), SDHC (show SDHC Antibodies), SDHD (show SDHD Antibodies), SDHAF1 and SDHAF2 (show Sdhaf2 Antibodies) have been identified over a decade.
Leukoencephalopathy with accumulated succinate is a key symptom of defective complex II assembly due to SDHAF1 mutations
Myocardial ischemic reperfusion injury in Sirt5 (show SIRT5 Antibodies)-/- heart is restored to wild-type levels by pretreatment with dimethyl malonate, a competitive inhibitor of succinate dehydrogenase (SDH (show SDHA Antibodies)), implicating alteration in SDH (show SDS Antibodies) activity as causative of the injury.
The succinate dehydrogenase (SDH) complex (or complex II) of the mitochondrial respiratory chain is composed of 4 individual subunits. The protein encoded by this gene resides in the mitochondria, and is essential for SDH assembly, but does not physically associate with the complex in vivo. Mutations in this gene are associated with SDH-defective infantile leukoencephalopathy (mitochondrial complex II deficiency).
succinate dehydrogenase complex assembly factor 1
, succinate dehydrogenase assembly factor 1, mitochondrial
, LYR motif-containing protein 8
, SDH assembly factor 1
, LYR motif containing 8