BCS1L Polyclonal antibody

BCS1L Polyclonal Antibody for WB, IF, IHC, ELISA

Host / Isotype

Rabbit / IgG

Reactivity

human, mouse, rat

Applications

WB, IF, IHC, ELISA

Conjugate

Unconjugated

Cat no : 10175-2-AP

Print datasheet

Synonyms

BCS1L, BJS, FLNMS, GRACILE, h BCS, h BCS1, Hs.6719, Mitochondrial chaperone BCS1, PTD



经过测试的应用

Positive WB detected inA549 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 inhuman 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 inA549 cells

推荐稀释比

ApplicationDilution
Western Blot (WB)WB : 1:500-1:1000
Immunohistochemistry (IHC)IHC : 1:20-1:200
Immunofluorescence (IF)/ICCIF/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

WBSee 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 ApplicationsWB
Tested Reactivity human, mouse, rat
Cited Reactivityhuman
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 NumberBC007500
Gene Symbol BCS1L
Gene ID (NCBI) 617
RRIDAB_2259019
Conjugate Unconjugated
Form Liquid
Purification MethodAntigen affinity purification
UNIPROT IDQ9Y276
Storage Buffer PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
Storage ConditionsStore 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-APDownload protocol
IHC protocol for BCS1L antibody 10175-2-APDownload protocol
IF protocol for BCS1L antibody 10175-2-APDownload protocol
Standard Protocols
Click here to view our Standard Protocols

发表文章

SpeciesApplicationTitle
humanWB

Sci Transl Med

Molecular diagnosis of infantile mitochondrial disease with targeted next-generation sequencing.

Authors - Sarah E Calvo
humanWB

Hum Mol Genet

Impaired complex III assembly associated with BCS1L gene mutations in isolated mitochondrial encephalopathy.

Authors - Fernandez-Vizarra Erika E
humanWB

Hum Mutat

Cellular pathophysiological consequences of BCS1L mutations in mitochondrial complex III enzyme deficiency.

Authors - Morán María M
humanWB

PLoS One

How do human cells react to the absence of mitochondrial DNA?

Authors - Mineri Rossana R
humanWB

Cell Rep

Distinct Roles of Mitochondrial HIGD1A and HIGD2A in Respiratory Complex and Supercomplex Biogenesis.

Authors - Alba Timón-Gómez
humanWB

Biochim Biophys Acta

LYRM7/MZM1L is a UQCRFS1 chaperone involved in the last steps of mitochondrial Complex III assembly in human cells.

Authors - Sánchez Ester E