{"title":"SDHAF","description":"\u003ch2 class=\"highlited\" data-mce-fragment=\"1\"\u003e\u003cspan data-mce-fragment=\"1\"\u003eAbout SDHAF \/ Succinate Dehydrogenase Complex Assembly Factor:\u003c\/span\u003e\u003c\/h2\u003e\n\u003cp data-mce-fragment=\"1\"\u003eSuccinate dehydrogenase complex assembly factor 1 (SDHAF1) is a chaperone protein used in the assembly of the succinate dehydrogenase (SDH) complex (complex II). It is also known as LYR motif-containing protein 8 (LYRM8).\u003cbr data-mce-fragment=\"1\"\u003eMutations in SDHAF1 are strongly linked with mitochondrial complex II deficiency. Although this is a disorder of the mitochondrial respiratory chain it has a wide range of psychomotor manifestations. SDHAF1 mutations are also strongly linked with SDH-defective infantile leukoencephalopathy.\u003c\/p\u003e\n\u003cp data-mce-fragment=\"1\"\u003e\u003cstrong data-mce-fragment=\"1\"\u003eSDHAF Function\u003c\/strong\u003e\u003cbr data-mce-fragment=\"1\"\u003eThe SDHAF1 protein shields SDHB from the oxidants which could otherwise damage it. This enables the assembly and stabilization of the succinate dehydrogenase (SDH) complex (complex II). SDH is necessary for both the tricarboxylic acid (TCA) cycle and the mitochondrial electron transport chain.\u003cbr data-mce-fragment=\"1\"\u003eIt also binds directly to the co-chaperone protein HSC20. This enables the scaffold protein ISCU to transfer the Fe-S prosthetic group to recipient apoproteins (namely SDHB). Indirectly, therefore, the SDHAF1 protein makes it possible for SDHB to acquire Fe-S clusters.\u003c\/p\u003e\n\u003cp data-mce-fragment=\"1\"\u003e\u003cstrong data-mce-fragment=\"1\"\u003eSuccinate Dehydrogenase Complex Assembly Factor Structure\u003c\/strong\u003e\u003cbr data-mce-fragment=\"1\"\u003eThe SDHAF1 gene is located in position 13.12 of the q arm of chromosome 19 (19q13.12). It has 1 exon. The SDHAF1 protein is a 12.8 kDa protein. It consists of 115 amino acids.\u003c\/p\u003e\n\u003cp data-mce-fragment=\"1\"\u003e\u003cstrong data-mce-fragment=\"1\"\u003eSDHAF Interaction\u003c\/strong\u003e\u003cbr data-mce-fragment=\"1\"\u003eSDHAF1 has a total of 27 protein-protein interactions. Of these, 15 are co-complex interactions. Genes known to interact with SDHAF1 include:\u003cbr data-mce-fragment=\"1\"\u003eccdc136\u003cbr data-mce-fragment=\"1\"\u003eCIDEB\u003cbr data-mce-fragment=\"1\"\u003eHSCB\u003cbr data-mce-fragment=\"1\"\u003eHSPA9\u003cbr data-mce-fragment=\"1\"\u003eISCU\u003cbr data-mce-fragment=\"1\"\u003eKRT27\u003cbr data-mce-fragment=\"1\"\u003eSDHB\u003c\/p\u003e\n\u003cp data-mce-fragment=\"1\"\u003e\u003cstrong data-mce-fragment=\"1\"\u003eSuccinate Dehydrogenase Complex Assembly Factor Mechanism of action\u003c\/strong\u003e\u003cbr data-mce-fragment=\"1\"\u003eAn arginine-rich region in the C terminus of SDHAF1 creates a transient bond with the aromatic peptides of SDHB. This engages specifically with an Fe-S donor complex through an LYR motif near its N-terminal domain. The scaffold is holo-ISCU, and the co-chaperone-chaperone pair is HSC20-HSPA9.\u003c\/p\u003e","products":[{"product_id":"sdhaf1-human","title":"SDHAF1 Human","description":"\u003cp\u003eSuccinate Dehydrogenase Complex Assembly Factor 1 Human Recombinant\u003c\/p\u003e\n\n\u003cdiv class=\"synonyms_hide\"\u003e\n  \u003cp\u003eSuccinate Dehydrogenase Complex Assembly Factor 1, LYR\nMotif-Containing Protein 8, LYR Motif\nContaining 8, SDH Assembly Factor 1, LYRM8, Succinate Dehydrogenase Assembly\nFactor 1, Mitochondrial, Succinate dehydrogenase assembly factor 1,\nmitochondrial.\u003c\/p\u003e\n\u003c\/div\u003e","brand":"prospecbio","offers":[{"title":"2µg","offer_id":43174097944660,"sku":"PRO-2123","price":60.0,"currency_code":"USD","in_stock":true},{"title":"10µg","offer_id":43174097977428,"sku":"PRO-2123","price":145.0,"currency_code":"USD","in_stock":true},{"title":"100µg","offer_id":43174098010196,"sku":"PRO-2123","price":1200.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0559\/3219\/0804\/files\/Prospecbio-sdhaf1-human-PRO-2123.png?v=1776445533"},{"product_id":"sdhaf2-human","title":"SDHAF2 Human","description":"\u003cp\u003eSuccinate Dehydrogenase Complex Assembly Factor 2 Human Recombinant\u003c\/p\u003e\n\n\u003cdiv class=\"synonyms_hide\"\u003e\n  \u003cp\u003eSuccinate dehydrogenase assembly factor 2 mitochondrial, SDH assembly factor 2, Succinate dehydrogenase subunit 5, mitochondrial, hSDH5, SDHAF2, C11orf79, PGL2, SDH5.\u003c\/p\u003e\n\u003c\/div\u003e","brand":"prospecbio","offers":[{"title":"5µg","offer_id":43174098731092,"sku":"PRO-593","price":60.0,"currency_code":"USD","in_stock":true},{"title":"20µg","offer_id":43174098763860,"sku":"PRO-593","price":145.0,"currency_code":"USD","in_stock":true},{"title":"1mg","offer_id":43174098796628,"sku":"PRO-593","price":2700.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0559\/3219\/0804\/files\/Prospecbio-sdhaf2-human-PRO-593.png?v=1776445537"}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0559\/3219\/0804\/collections\/Image_53abf0f5-2867-4cb8-8acd-f96f03d88ccd.png?v=1700647873","url":"https:\/\/prospecbio.com\/collections\/sdhaf.oembed","provider":"prospecbio","version":"1.0","type":"link"}