The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
补充说明Protein concentration is above or equal to 0.05 mg/ml. Best used within three months from the date of receipt.
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Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00 Constituents: 0.3% Glutathione, 0.79% Tris HCl
COX15 homolog, cytochrome c oxidase assembly protein
COX15, S. cerevisiae, homolog of
cytochrome c oxidase assembly homolog 15 (yeast)
Cytochrome c oxidase assembly protein COX15 homolog
cytochrome c oxidase subunit 15
功能May be involved in the biosynthesis of heme A.
组织特异性Predominantly found in tissues characterized by high rates of oxidative phosphorylation (OxPhos), including muscle, heart, and brain.
通路Porphyrin metabolism; heme A biosynthesis; heme A from heme O: step 1/1.
疾病相关Defects in COX15 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. Defects in COX15 are a cause of Leigh syndrome (LS) [MIM:256000]. An early-onset progressive neurodegenerative disorder characterized by the presence of focal, bilateral lesions in one or more areas of the central nervous system including the brainstem, thalamus, basal ganglia, cerebellum and spinal cord. Clinical features depend on which areas of the central nervous system are involved and include subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, and dysphagia.