| Объем | 50 мкг |
| Синонимы | Cytochrome c oxidase subunit 6B1 (Cytochrome c oxidase subunit VIb isoform 1) (COX VIb-1), COX6B1, COX6B |
| Клональность | Polyclonal Antibody |
| Организм | Human |
| uniprot | P14854 |
| Иммуноген | Recombinant Human Cytochrome c oxidase subunit 6B1 protein (1-86AA) |
| Источник | Rabbit |
| Видовая специфичность | Human |
| Применение | ELISA, IF, Recommended dilution: IF:1:50-1:200 |
| Примечание | 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. |
| Клональность1 | Polyclonal |
| Изотип | IgG |
| Коньюгат | Non-conjugated |
| Буффер | Preservative: 0.03% Proclin 300<br />Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
| Форма | Liquid |
| Хранение | Upon receipt, store at -20°C or -80°C. Avoid repeated freeze. |
| Метод очистки | >95%, Protein G purified |
| Абревеатура | Cytochrome c oxidase subunit 6B1 protein |
| Области исследований | Cancer, Tags & Cell Markers, Metabolism, Signal transduction |
| Ссылка на страницу на сайте производителя | ссылка |
Immunofluorescent analysis of A549 cells using CSB-PA00395A0Rb at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
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Наименование: COX6B1 Antibody.