The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 1 - 5 µg/ml. Predicted molecular weight: 10 kDa.
Use a concentration of 0.3 µg/ml.
FunctionConnects the two COX monomers into the physiological dimeric form.
Involvement in diseaseDefects 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.
Sequence similaritiesBelongs to the cytochrome c oxidase subunit 6B family.
ab54575 at 0.3 µg/ml staining Cytochrome C Oxidase subunit VIb in human kidney tissue section by Immunohistochemistry (Formalin/ PFA fixed paraffin-embedded sections).
References for Anti-Cytochrome C Oxidase subunit VIb antibody (ab54575)
This product has been referenced in:
Nuss JE et al. Multi-Faceted Proteomic Characterization of Host Protein Complement of Rift Valley Fever Virus Virions and Identification of Specific Heat Shock Proteins, Including HSP90, as Important Viral Host Factors. PLoS One9:e93483 (2014).
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