验证数据展示
经过测试的应用
| Positive WB detected in | A549 cells, HEK-293 cells, HeLa cells, human brain tissue, mouse colon tissue, mouse kidney tissue, mouse liver tissue, mouse skeletal muscle tissue, mouse small intestine tissue, rat liver tissue |
| Positive IHC detected in | human gliomas tissue, human kidney tissue Note: suggested antigen retrieval with TE buffer pH 9.0; (*) Alternatively, antigen retrieval may be performed with citrate buffer pH 6.0 |
| Positive IF/ICC detected in | A549 cells |
推荐稀释比
| Application | Dilution |
|---|---|
| Western Blot (WB) | WB : 1:500-1:1000 |
| Immunohistochemistry (IHC) | IHC : 1:20-1:200 |
| Immunofluorescence (IF)/ICC | IF/ICC : 1:20-1:200 |
| It is recommended that this reagent should be titrated in each testing system to obtain optimal results. | |
| Sample-dependent, Check data in validation data gallery. | |
发表文章中的应用
| WB | See 7 publications below |
产品信息
10175-2-AP targets BCS1L in WB, IF, IHC, ELISA applications and shows reactivity with human, mouse, rat samples.
| Tested Applications | WB, IF, IHC, ELISA Application Description |
| Cited Applications | WB |
| Tested Reactivity | human, mouse, rat |
| Cited Reactivity | human |
| Immunogen | BCS1L fusion protein Ag0231 种属同源性预测 |
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Full Name | BCS1-like (yeast) |
| Synonyms | BCS1L, BJS, FLNMS, GRACILE, h BCS, h BCS1, Hs.6719, Mitochondrial chaperone BCS1, PTD |
| Calculated Molecular Weight | 48 kDa |
| Observed Molecular Weight | 50-55 kDa |
| GenBank Accession Number | BC007500 |
| Gene Symbol | BCS1L |
| Gene ID (NCBI) | 617 |
| RRID | AB_2259019 |
| Conjugate | Unconjugated |
| Form | Liquid |
| Purification Method | Antigen affinity purification |
| UNIPROT ID | Q9Y276 |
| Storage Buffer | PBS with 0.02% sodium azide and 50% glycerol pH 7.3. |
| Storage Conditions | Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage. |
背景介绍
Human BCS1-like (BCS1L), a mitochondrial inner-membrane protein, is a chaperone necessary for the assembly of mitochondrial respiratory chain complex III. Recently studies indicated that the mutations in this protein may cause the GRACILE (growth retardation, aminoaciduria, cholestasis, iron overload, lactacidosis, and early death) syndrome, a recessively inherited lethal disease characterized by fetal growth retardation, lactic acidosis, aminoaciduria, cholestasis, and abnormalities in iron metabolism.
实验方案
| Product Specific Protocols | |
|---|---|
| WB protocol for BCS1L antibody 10175-2-AP | Download protocol |
| IHC protocol for BCS1L antibody 10175-2-AP | Download protocol |
| IF protocol for BCS1L antibody 10175-2-AP | Download protocol |
| Standard Protocols | |
|---|---|
| Click here to view our Standard Protocols |
发表文章
| Species | Application | Title |
|---|---|---|
Sci Transl Med Molecular diagnosis of infantile mitochondrial disease with targeted next-generation sequencing. | ||
Hum Mol Genet Impaired complex III assembly associated with BCS1L gene mutations in isolated mitochondrial encephalopathy. | ||
Hum Mutat Cellular pathophysiological consequences of BCS1L mutations in mitochondrial complex III enzyme deficiency. | ||
Cell Rep Distinct Roles of Mitochondrial HIGD1A and HIGD2A in Respiratory Complex and Supercomplex Biogenesis. | ||
Biochim Biophys Acta LYRM7/MZM1L is a UQCRFS1 chaperone involved in the last steps of mitochondrial Complex III assembly in human cells. |
