
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)
COX6B1 Antibody
CSB-PA00395A0RB
ApplicationsImmunoFluorescence, ELISA
Product group Antibodies
TargetCOX6B1
Overview
- SupplierCusabio
- Product NameCOX6B1 Antibody
- Delivery Days Customer20
- ApplicationsImmunoFluorescence, ELISA
- CertificationResearch Use Only
- ClonalityPolyclonal
- ConjugateUnconjugated
- Gene ID1340
- Target nameCOX6B1
- Target descriptioncytochrome c oxidase subunit 6B1
- Target synonymsCOX VIb-1; COX6B; COXG; COXVIb1; cytochrome c oxidase subunit 6B1; cytochrome c oxidase subunit VIb polypeptide 1 (ubiquitous); MC4DN7
- HostRabbit
- IsotypeIgG
- Protein IDP14854
- Protein NameCytochrome c oxidase subunit 6B1
- Scientific DescriptionDefects 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.
- Storage Instruction-20°C or -80°C
- UNSPSC12352203