Cusabio Polyclonal Antibodies
COX6B1 Antibody | CSB-PA00395A0Rb
- SKU:
- CSB-PA00395A0Rb
- Availability:
- 3 to 7 Working Days
Description
COX6B1 Antibody | CSB-PA00395A0Rb | Cusabio
COX6B1 Antibody is Available at Gentaur Genprice with the fastest delivery.
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Product Type: Polyclonal Antibody
Target Names: COX6B1
Aliases: Cytochrome c oxidase subunit 6B1 (Cytochrome c oxidase subunit VIb isoform 1) (COX VIb-1), COX6B1, COX6B
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.
Isotype: IgG
Conjugate: Non-conjugated
Clonality: Polyclonal
Uniport ID: P14854
Host Species: Rabbit
Species Reactivity: Human
Immunogen: Recombinant Human Cytochrome c oxidase subunit 6B1 protein (1-86AA)
Immunogen Species: Human
Applications: ELISA, IF
Tested Applications: ELISA, IF; Recommended dilution: IF:1:50-1:200
Purification Method: >95%, Protein G purified
Dilution Ratio1: ELISA:1:2000-1:10000
Dilution Ratio2: IF:1:50-1:200
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Buffer: Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Form: Liquid
Storage: Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Initial Research Areas: Tags & Cell Markers
Research Areas: Cancer;Tags & Cell Markers;Metabolism;Signal transduction