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 StabilityStore product at 4°C if to be used immediately within two weeks.
For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C.
Aliquots are stable at -20°C for 12 months after receipt.
Dilute required amount only prior to immediate use.
Further dilutions can be made in assay buffer.
Caution: FITC conjugates are sensitive to light.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ug
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by Protein G affinity chromatography.
Form：Supplied as a liquid in PBS. Labeled with Fluorescein isothiocyanate (FITC).
Specificity：Recognizes human COX6B1. Species Crossreactivity: mouse, rat, bovine
Isotype：IgG
Calc Applications Abbrev：E WB
Calc Crossreactivity：Hu Rt
Immunogen：Recombinant human Cytochrome c oxidase subunit 6B1 protein
