COX6B1 antibody (AA 1-86)
Quick Overview for COX6B1 antibody (AA 1-86) (ABIN7149179)
Target
See all COX6B1 AntibodiesReactivity
Host
Clonality
Conjugate
Application
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Binding Specificity
- AA 1-86
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Cross-Reactivity
- Human
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Purification
- >95%, Protein G purified
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Immunogen
- Recombinant Human Cytochrome c oxidase subunit 6B1 protein (1-86AA)
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Isotype
- IgG
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Application Notes
- Recommended dilution: IF:1:50-1:200,
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Restrictions
- For Research Use only
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Format
- Liquid
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Buffer
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Preservative: 0.03 % Proclin 300
Constituents: 50 % Glycerol, 0.01M PBS, PH 7.4 -
Preservative
- ProClin
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Precaution of Use
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Storage
- -20 °C,-80 °C
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Storage Comment
- Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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- COX6B1 (Cytochrome C Oxidase Subunit VIb Polypeptide 1 (Ubiquitous) (COX6B1))
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Alternative Name
- COX6B1
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Background
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Background: 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.
Aliases: COX 6B antibody, COX VIb 1 antibody, COX VIb-1 antibody, COX6B antibody, COX6B1 antibody, COXG antibody, COXVIb1 antibody, CX6B1_HUMAN antibody, Cytochrome c oxidase subunit 6B1 antibody, Cytochrome c oxidase subunit VIb antibody, Cytochrome c oxidase subunit VIb isoform 1 antibody, cytochrome c oxidase subunit VIb polypeptide 1 (ubiquitous) antibody, Cytochrome c oxidase subunit Vib polypeptide 1 antibody
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UniProt
- P14854
Target
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