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Antibody Product Overview
| ELK.No | ES18080 |
| Product name | BCS1 rabbit pAb |
| Reactivity | Human; Mouse |
| Applications | WB |
| Other name | |
| Size | 50μL |
| Unit price ($) | 148 |
| Human gene ID | 617 |
| Human Swiss-Prot | Q9Y276 |
| Source | Rabbit |
| Isotype | IgG |
| Target | BCS1 |
| Fields | |
| Gene name | BCS1L BCS1 |
| Protein name | BCS1 |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 66821 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | Q9CZP5 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | |
| Rat gene link | |
| Rat Swiss-Prot | |
| Rat Swiss link | |
| Immunogen | Synthesized peptide derived from human BCS1 AA range: 29-79 |
| Specificity | This antibody detects endogenous levels of BCS1 at Human/Mouse |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Clonality | Polyclonal |
| Dilution | WB 1：500-2000 |
| Purification | The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | 46kD |
| Observed band (KD) | |
| Background | This gene encodes a homolog of the S. cerevisiae bcs1 protein which is involved in the assembly of complex III of the mitochondrial respiratory chain. The encoded protein does not contain a mitochondrial targeting sequence but experimental studies confirm that it is imported into mitochondria. Mutations in this gene are associated with mitochondrial complex III deficiency and the GRACILE syndrome. Several alternatively spliced transcripts encoding two different isoforms have been described. [provided by RefSeq, Jan 2016], |
| Function | disease:Defects in BCS1L are a cause of mitochondrial complex III deficiency (CIII deficiency) [MIM:124000]. CIII deficiency is characterized by congenital lactic acidosis. Patients had severe failure to thrive, liver dysfunction and renal tubulopathy.,disease:Defects in BCS1L are the cause of Bjoernstad syndrome (BJS) [MIM:262000]. BJS is an autosomal recessive condition characterized by sensorineural hearing loss and pili torti. The hearing loss in BJS is congenital and of variable severity. Pili torti (twisted hairs), a condition in which the hair shafts are flattened at irregular intervals and twisted 180 degrees from the normal axis, making the hair extremely brittle, is usually recognized early in childhood.,disease:Defects in BCS1L are the cause of GRACILE syndrome [MIM:603358]. GRACILE stands for 'growth retardation, aminoaciduria, cholestasis, iron overload, lactic acidosis, and |
| Subcellular location | Mitochondrion inner membrane ; Single-pass membrane protein . |
| Expression | Ubiquitous. |
Additional Images
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Western blot analysis of lysates from 3T3 cells, primary antibody was diluted at 1:1000, 4°over night
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: AO-06-ES18080-50
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