Cytochrome c oxidase subunit 6B1 (Biotin)

Size

£460.00

SKU:: 312819
Purity:: Purified by Protein G affinity chromatography.
Storage Conditions:: -20°C
Supplier:: United States Biological
Host:: Rabbit
Immunogen:: Recombinant human Cytochrome c oxidase subunit 6B1 protein
Format:: Supplied as a liquid in PBS. Labeled with Biotin
Extra Details:: Defects in COX6B1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:220110], also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome. Applications: Suitable for use in ELISA and Western Blot. Other applications not tested. Recommended Dilution: Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.