BCS1L Polyclonal antibody

BCS1L Polyclonal Antibody for IF, IHC, WB, ELISA

Host / Isotype

Rabbit / IgG

Reactivity

human, mouse, rat

Applications

WB, IHC, IF, ELISA

Conjugate

Unconjugated

Cat no : 10175-2-AP

Synonyms

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



Tested Applications

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 detected inA549 cells

Recommended dilution

ApplicationDilution
Western Blot (WB)WB : 1:500-1:1000
Immunohistochemistry (IHC)IHC : 1:20-1:200
Immunofluorescence (IF)IF : 1:20-1:200
Sample-dependent, check data in validation data gallery

Published Applications

WBSee 7 publications below

Product Information

10175-2-AP targets BCS1L in WB, IHC, IF, ELISA applications and shows reactivity with human, mouse, rat samples.

Tested Reactivity human, mouse, rat
Cited Reactivity human
Host / Isotype Rabbit / IgG
Class Polyclonal
Type Antibody
Immunogen BCS1L fusion protein Ag0231
Full Name BCS1-like (yeast)
Calculated molecular weight 48 kDa
Observed molecular weight 50-55 kDa
GenBank accession numberBC007500
Gene symbol BCS1L
Gene ID (NCBI) 617
Conjugate Unconjugated
Form Liquid
Purification Method Antigen affinity purification
Storage Buffer PBS with 0.1% 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.

Background Information

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.

Protocols

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

Publications

SpeciesApplicationTitle
humanWB

Biochim Biophys Acta

Assembly of the oxidative phosphorylation system in humans: what we have learned by studying its defects.

Authors - Fernández-Vizarra Erika E
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
humanWB

Cell Rep

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

Authors - Alba Timón-Gómez
humanWB

PLoS One

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

Authors - Mineri Rossana R
humanWB

Hum Mutat

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

Authors - Morán María M
humanWB

Hum Mol Genet

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

Authors - Fernandez-Vizarra Erika E