BCS1L Polyclonal antibody

Name: BCS1L Polyclonal antibody
Brand: Proteintech
SKU: 10175-2-AP

BCS1L Polyclonal Antibody for WB, IF, IHC, ELISA

Host / Isotype

Rabbit / IgG

Reactivity

human, mouse, rat

Applications

WB, IF, IHC, ELISA

Conjugate

Unconjugated

Publications

Review Product

Cat no : 10175-2-AP

Synonyms

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

Validation Data Gallery

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AllWBIHCIF

WB analysis of A549 using 10175-2-AP
A549 cells were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:1000 incubated at room temperature for 1.5 hours.
WB analysis of human brain using 10175-2-AP
human brain tissue were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:500 incubated at room temperature for 1.5 hours.
WB analysis of HeLa using 10175-2-AP
HeLa cells were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:1000 incubated at room temperature for 1.5 hours.
WB analysis of mouse small intestine using 10175-2-AP
mouse small intestine tissue were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:600 incubated at room temperature for 1.5 hours.
WB analysis of mouse kidney using 10175-2-AP
mouse kidney tissue were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:500 incubated at room temperature for 1.5 hours.
WB analysis of rat liver using 10175-2-AP
rat liver tissue were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:1000 incubated at room temperature for 1.5 hours.
WB analysis of mouse liver using 10175-2-AP
mouse liver tissue were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:1000 incubated at room temperature for 1.5 hours.
WB analysis of mouse colon using 10175-2-AP
mouse colon tissue were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:1000 incubated at room temperature for 1.5 hours.
WB analysis of mouse skeletal muscle using 10175-2-AP
mouse skeletal muscle tissue were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:1000 incubated at room temperature for 1.5 hours.
WB analysis of HEK-293 using 10175-2-AP
HEK-293 cells were subjected to SDS PAGE followed by western blot with 10175-2-AP (BCS1L antibody) at dilution of 1:1000 incubated at room temperature for 1.5 hours.
IHC staining of human gliomas using 10175-2-AP
Immunohistochemical analysis of paraffin-embedded human gliomas using 10175-2-AP (BCS1L antibody) at dilution of 1:50 (under 10x lens).
IHC staining of human kidney using 10175-2-AP
Immunohistochemical analysis of paraffin-embedded human kidney using 10175-2-AP (BCS1L antibody) at dilution of 1:50 (under 10x lens).
IHC staining of human kidney using 10175-2-AP
Immunohistochemical analysis of paraffin-embedded human kidney using 10175-2-AP (BCS1L antibody) at dilution of 1:50 (under 40x lens).
IF Staining of A549 using 10175-2-AP
Immunofluorescent analysis of (10% Formaldehyde) fixed A549 cells using 10175-2-AP (BCS1L antibody) at dilution of 1:50 and Alexa Fluor 488-conjugated Goat Anti-Rabbit IgG(H+L).

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Cat No. 10175-2-AP

Size/Concentration

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テクニカルサポートまたはご購入後1年間の交換／補填対応を承ります。詳細はこちらをご覧ください。

Tested Applications

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

Recommended dilution

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.

Published Applications

WB	See 7 publications below

Product Information

10175-2-AP targets BCS1L in WB, IF, IHC, 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 相同性解析による交差性が予測される生物種
Full Name	BCS1-like (yeast)
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
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 -20^oC 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-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

Publications

Species	Application	Title
human	WB	Sci Transl Med Molecular diagnosis of infantile mitochondrial disease with targeted next-generation sequencing. Authors - Sarah E Calvo View Article
human	WB	Hum Mol Genet Impaired complex III assembly associated with BCS1L gene mutations in isolated mitochondrial encephalopathy. Authors - Fernandez-Vizarra Erika E View Article
human	WB	Hum Mutat Cellular pathophysiological consequences of BCS1L mutations in mitochondrial complex III enzyme deficiency. Authors - Morán María M View Article
human	WB	PLoS One How do human cells react to the absence of mitochondrial DNA? Authors - Mineri Rossana R View Article
human	WB	Cell Rep Distinct Roles of Mitochondrial HIGD1A and HIGD2A in Respiratory Complex and Supercomplex Biogenesis. Authors - Alba Timón-Gómez View Article
human	WB	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 View Article

Target

Host

Species Reactivity

Species Reactivity

Conjugate

Conjugate

BCS1L Polyclonal antibody

BCS1L Polyclonal Antibody for WB, IF, IHC, ELISA

Validation Data Gallery

WB analysis of A549 using 10175-2-AP

WB analysis of human brain using 10175-2-AP

WB analysis of HeLa using 10175-2-AP

WB analysis of mouse small intestine using 10175-2-AP

WB analysis of mouse kidney using 10175-2-AP

WB analysis of rat liver using 10175-2-AP

WB analysis of mouse liver using 10175-2-AP

WB analysis of mouse colon using 10175-2-AP

WB analysis of mouse skeletal muscle using 10175-2-AP

WB analysis of HEK-293 using 10175-2-AP

IHC staining of human gliomas using 10175-2-AP

IHC staining of human kidney using 10175-2-AP

IHC staining of human kidney using 10175-2-AP

IF Staining of A549 using 10175-2-AP

Tested Applications

Recommended dilution

Published Applications

Product Information

Background Information

Protocols

Publications

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

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

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

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

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

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